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Stephen Tint

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Quan Shang; Luxing Pan; Monica Saumoy; John Y L Chiang; Stephen G Tint; Gerald Salen; Guorong Xu
An overlapping binding site in the CYP7A1 promoter allows activation of FXR to override the stimulation by LXRalpha.
Curzio Solcà; Bhaswati Pandit; Hongwei Yu; Stephen G Tint; Shailendra Patel
Loss of apolipoprotein E exacerbates the neonatal lethality of the Smith-Lemli-Opitz syndrome mouse.
Hongwei Yu; Bassam Wakim; Man Li; Brian Halligan; Stephen G Tint; Shailendra Patel
Quantifying raft proteins in neonatal mouse brain by 'tube-gel' protein digestion label-free shotgun proteomics.

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2007: Quan Shang; Luxing Pan; Monica Saumoy; John Y L Chiang; Stephen G Tint; Gerald Salen; Guorong Xu
An overlapping binding site in the CYP7A1 promoter allows activation of FXR to override the stimulation by LXRalpha.
American journal of physiology. Gastrointestinal and liver physiology 2007;293(4):G817-23.
2007: Curzio Solcà; Bhaswati Pandit; Hongwei Yu; Stephen G Tint; Shailendra Patel
Loss of apolipoprotein E exacerbates the neonatal lethality of the Smith-Lemli-Opitz syndrome mouse.
Molecular genetics and metabolism 2007;91(1):7-14.
2007: Hongwei Yu; Bassam Wakim; Man Li; Brian Halligan; Stephen G Tint; Shailendra Patel
Quantifying raft proteins in neonatal mouse brain by 'tube-gel' protein digestion label-free shotgun proteomics.
Proteome science 2007;5():17.
2007: Hongwei Yu; Man Li; Stephen G Tint; Jianliang Chen; Guorong Xu; Shailendra Patel
Selective reconstitution of liver cholesterol biosynthesis promotes lung maturation but does not prevent neonatal lethality in Dhcr7 null mice.
BMC developmental biology 2007;7():27.
2006: Heng-Ling Liou; Sayali S Dixit; Sujuan Xu; Stephen G Tint; Ann M Stock; Peter Lobel
NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols.
The Journal of biological chemistry 2006;281(48):36710-23.
2006: Stephen G Tint; Hongwei Yu; Quan Shang; Guorong Xu; Shailendra Patel
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
Journal of lipid research 2006;47(7):1535-41.
2006: James W Brodsky; Andrew D Beischer; Dip Anat; Cara East; Elizabeth Soltero; Stephen G Tint; Gerald Salen; Julie Silverman
Cerebrotendinous xanthomatosis: a rare cause of bilateral Achilles tendon swelling and ataxia. A case report.
The Journal of bone and joint surgery. American volume 2006;88(6):1340-4.
2006: Quan Shang; Luxing Pan; Monica Saumoy; John Y L Chiang; Stephen G Tint; Gerald Salen; Guorong Xu
The stimulatory effect of LXRalpha is blocked by SHP despite the presence of a LXRalpha binding site in the rabbit CYP7A1 promoter.
Journal of lipid research 2006;47(5):997-1004.
2006: Thomas N Tulenko; Kathy Boeze-Battaglia; R Preston Mason; Stephen G Tint; Robert D Steiner; William E Connor; Edward F Labelle
A membrane defect in the pathogenesis of the Smith-Lemli-Opitz syndrome.
Journal of lipid research 2006;47(1):134-43.
2005: Hongwei Yu; Andy Wessels; Stephen G Tint; Shailendra Patel
Partial rescue of neonatal lethality of Dhcr7 null mice by a nestin promoter-driven DHCR7 transgene expression.
Brain research. Developmental brain research 2005;156(1):46-60.
2005: Akira Honda; Gerald Salen; Yasushi Matsuzaki; Ashok K Batta; Guorong Xu; Takeshi Hirayama; Stephen G Tint; Mikio Doy; Sarah Shefer
Disrupted coordinate regulation of farnesoid X receptor target genes in a patient with cerebrotendinous xanthomatosis.
Journal of lipid research 2005;46(2):287-96.
2005: Hai Li; Frank Chen; Quan Shang; Luxing Pan; Benjamin L Shneider; John Y L Chiang; Barry M Forman; Meenakshisundaram Ananthanarayanan; Stephen G Tint; Gerald Salen; Guorong Xu
FXR-activating ligands inhibit rabbit ASBT expression via FXR-SHP-FTF cascade.
American journal of physiology. Gastrointestinal and liver physiology 2005;288(1):G60-6.
2004: Mark Merris; Jessica Kraeft; Stephen G Tint; John Lenard
Long-term effects of sterol depletion in C. elegans: sterol content of synchronized wild-type and mutant populations.
Journal of lipid research 2004;45(11):2044-51.
2004: Hai Li; Guorong Xu; Quan Shang; Luxing Pan; Sarah Shefer; Ashok K Batta; Jaya Bollineni; Stephen G Tint; Brad T Keller; Gerald Salen
Inhibition of ileal bile acid transport lowers plasma cholesterol levels by inactivating hepatic farnesoid X receptor and stimulating cholesterol 7 alpha-hydroxylase.
Metabolism: clinical and experimental 2004;53(7):927-32.
2004: Ashok K Batta; Gerald Salen; Stephen G Tint
Hydrophilic 7 beta-hydroxy bile acids, lovastatin, and cholestyramine are ineffective in the treatment of cerebrotendinous xanthomatosis.
Metabolism: clinical and experimental 2004;53(5):556-62.
2004: Guorong Xu; Lu-Xing Pan; Hai Li; Quan Shang; Akira Honda; Sarah Shefer; Jaya Bollineni; Yasushi Matsuzaki; Stephen G Tint; Gerald Salen
Dietary cholesterol stimulates CYP7A1 in rats because farnesoid X receptor is not activated.
American journal of physiology. Gastrointestinal and liver physiology 2004;286(5):G730-5.
2004: David E Sleat; Jennifer A Wiseman; Mukarram El-Banna; Sandy M Price; Lucie Verot; Michael M Shen; Stephen G Tint; marie T vanier; Steven U Walkley; Peter Lobel
Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(16):5886-91.
2004: Hongwei Yu; Andy Wessels; Jianliang Chen; Aimee L Phelps; John E Oatis; Stephen G Tint; Shailendra Patel
Late gestational lung hypoplasia in a mouse model of the Smith-Lemli-Opitz syndrome.
BMC developmental biology 2004;4():1.
2003: Heather G Waage-Baudet; Jean M Lauder; Deborah B Dehart; Kimberly D Kluckman; S Hiller; Stephen G Tint; Kathleen K Sulik
Abnormal serotonergic development in a mouse model for the Smith-Lemli-Opitz syndrome: implications for autism.
International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience 2003;21(8):451-9.
2003: C Mueller; S Patel; Mira Irons; K Antshel; G Salen; Stephen G Tint; Carolyn Bay
Normal cognition and behavior in a Smith-Lemli-Opitz syndrome patient who presented with Hirschsprung disease.
American journal of medical genetics. Part A 2003;123A(1):100-6.
2003: Guorong Xu; Hai Li; Lu-Xing Pan; Quan Shang; Akira Honda; Meenakshisundaram Ananthanarayanan; Sandra K Erickson; Benjamin L Shneider; Sarah Shefer; Jaya Bollineni; Barry M Forman; Yasushi Matsuzaki; Frederick J Suchy; Stephen G Tint; Gerald Salen
FXR-mediated down-regulation of CYP7A1 dominates LXRalpha in long-term cholesterol-fed NZW rabbits.
Journal of lipid research 2003;44(10):1956-62.
2003: Suzana Petanceska; Steven DeRosa; Ali Sharma; Nichole Diaz; karen duff; Stephen G Tint; Lorenzo M Refolo; Miguel Pappolla
Changes in apolipoprotein E expression in response to dietary and pharmacological modulation of cholesterol.
Journal of molecular neuroscience : MN 2003;20(3):395-406.
2002: Guorong Xu; Lu-Xing Pan; Hai Li; Barry M Forman; Sandra K Erickson; Sarah Shefer; Jaya Bollineni; Ashok K Batta; Jennifer Christie; Tsu-Hong Wang; John Michel; Steve Yang; Richard Tsai; Lily Lai; Kohei Shimada; Stephen G Tint; Gerald Salen
Regulation of the farnesoid X receptor (FXR) by bile acid flux in rabbits.
The Journal of biological chemistry 2002;277(52):50491-6.
2002: Ashok K Batta; Gerald Salen; Priti Batta; Stephen G Tint; David S Alberts; David L Earnest
Simultaneous quantitation of fatty acids, sterols and bile acids in human stool by capillary gas-liquid chromatography.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences 2002;775(2):153-61.
2002: WT O'Brien; Guorong Xu; AK Batta; Stephen G Tint; Gerald Salen; Charissa A Dyer; A Kendler; RJ Servatius
Developmental sensitivity of associative learning to cholesterol synthesis inhibitors.
Behavioural brain research 2002;129(1-2):141-52.
2002: Guorong Xu; Lu-Xing Pan; Sandra K Erickson; Barry M Forman; Benjamin L Shneider; Meenakshisundaram Ananthanarayanan; Xiaogui Li; Sarah Shefer; Natarajan Balasubramanian; Lin Ma; Hitoshi Asaoka; Steven R Lear; Lien B Nguyen; Isabelle Dussault; Frederick J Suchy; Stephen G Tint; Gerald Salen
Removal of the bile acid pool upregulates cholesterol 7alpha-hydroxylase by deactivating FXR in rabbits.
Journal of lipid research 2002;43(1):45-50.
2001: Lorenzo M Refolo; Miguel Pappolla; John LaFrancois; Brian Malester; Stephen D Schmidt; T Thomas-Bryant; Stephen G Tint; Runsheng Wang; Marc Mercken; Suzana Petanceska; karen duff
A cholesterol-lowering drug reduces beta-amyloid pathology in a transgenic mouse model of Alzheimer's disease.
Neurobiology of disease 2001;8(5):890-9.
2001: Lien B Nguyen; Gerald Salen; Sarah Shefer; Stephen G Tint; F Ruiz
Macrophage 3-hydroxy-3-methylglutaryl coenzyme a reductase activity in sitosterolemia: effects of increased cellular cholesterol and sitosterol concentrations.
Metabolism: clinical and experimental 2001;50(10):1224-9.
2001: Akira Honda; Gerald Salen; Yasushi Matsuzaki; Ashok K Batta; G Xu; E Leitersdorf; Stephen G Tint; Sandra K Erickson; Naomi Tanaka; Sarah Shefer
Side chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis.
The Journal of biological chemistry 2001;276(37):34579-85.
2001: Barbara U Fitzky; Fabian F Moebius; H Asaoka; Heather G Waage-Baudet; L Xu; G Xu; Nobuyo Maeda; Kimberly D Kluckman; S Hiller; H Yu; Ashok K Batta; Sarah Shefer; T Chen; G Salen; Kathleen K Sulik; R D Simoni; G C Ness; Hartmut Glossmann; Shailendra Patel; Stephen G Tint
7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome.
The Journal of clinical investigation 2001;108(6):905-15.
2001: Guorong Xu; Gerald Salen; Sarah Shefer; Stephen G Tint; Lien B Nguyen; Ashok K Batta; M Pcolinsky
Plant stanol fatty acid esters inhibit cholesterol absorption and hepatic hydroxymethyl glutaryl coenzyme A reductase activity to reduce plasma levels in rabbits.
Metabolism: clinical and experimental 2001;50(9):1106-12.
2001: Guorong Xu; Gerald Salen; Benjamin L Shneider; Meenakshisundaram Ananthanarayanan; Sarah Shefer; L Ma; Ashok K Batta; Lien B Nguyen; J J Lingutla; Stephen G Tint; M Pcolinsky; Frederick J Suchy
Cholecystectomy prevents expansion of the bile acid pool and inhibition of cholesterol 7alpha-hydroxylase in rabbits fed cholesterol.
Journal of lipid research 2001;42(9):1438-43.
2001: K Baudry; Evelyn Swain; Alain Rahier; Melody Germann; A Batta; S Rondet; S Mandala; K Henry; Stephen G Tint; Thomas D Edlind; M Kurtz; Joseph T Nickels
The effect of the erg26-1 mutation on the regulation of lipid metabolism in Saccharomyces cerevisiae.
The Journal of biological chemistry 2001;276(16):12702-11.
2001: Akira Honda; Gerald Salen; Yasushi Matsuzaki; Ashok K Batta; G Xu; E Leitersdorf; Stephen G Tint; Sandra K Erickson; Naomi Tanaka; Sarah Shefer
Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27(-/-) mice and CTX.
Journal of lipid research 2001;42(2):291-300.
2001: Lien B Nguyen; Sarah Shefer; Gerald Salen; Stephen G Tint; F Ruiz; J Bullock
Mechanisms for cholesterol homeostasis in rat jejunal mucosa: effects of cholesterol, sitosterol, and lovastatin.
Journal of lipid research 2001;42(2):195-200.
2000: G Salen; G Xu; Stephen G Tint; A K Batta; S Shefer
Hyperabsorption and retention of campestanol in a sitosterolemic homozygote: comparison with her mother and three control subjects.
Journal of lipid research 2000;41(11):1883-9.
2000: Sarah Shefer; Stephen G Tint; Danielle Jean-Guillaume; Evgueni Daikhin; A Kendler; Lien B Nguyen; Marc Yudkoff; Charissa A Dyer
Is there a relationship between 3-hydroxy-3-methylglutaryl coenzyme a reductase activity and forebrain pathology in the PKU mouse?
Journal of neuroscience research 2000;61(5):549-63.
2000: Lorenzo M Refolo; Brian Malester; John LaFrancois; Tara Bryant-Thomas; Runsheng Wang; Stephen G Tint; Kumar Sambamurti; karen duff; Miguel Pappolla
Hypercholesterolemia accelerates the Alzheimer's amyloid pathology in a transgenic mouse model.
Neurobiology of disease 2000;7(4):321-31.
2000: M Honda; Stephen G Tint; Akira Honda; Gerald Salen; Sarah Shefer; Ashok K Batta; Yasushi Matsuzaki; Naomi Tanaka
Regulation of cholesterol biosynthetic pathway in patients with the Smith-Lemli-Opitz syndrome.
Journal of inherited metabolic disease 2000;23(5):464-74.
2000: D Holmes Morton; G Salen; Ashok K Batta; Sarah Shefer; Stephen G Tint; D Belchis; B Shneider; Erik G Puffenberger; Laura N Bull; A S Knisely
Abnormal hepatic sinusoidal bile acid transport in an Amish kindred is not linked to FIC1 and is improved by ursodiol.
Gastroenterology 2000;119(1):188-95.
2000: Hongwei Yu; M H Lee; L Starck; Ellen R Elias; Mira Irons; Gerald Salen; Shailendra Patel; Stephen G Tint
Spectrum of Delta(7)-dehydrocholesterol reductase mutations in patients with the Smith-Lemli-Opitz (RSH) syndrome.
Human molecular genetics 2000;9(9):1385-91.
2000: WT O'Brien; Guorong Xu; Stephen G Tint; Gerald Salen; RJ Servatius
Blocking cholesterol synthesis impairs acquisition of the classically conditioned eyeblink response.
Integrative physiological and behavioral science : the official journal of the Pavlovian Society 2000;35(2):120-31.
2000: Leesa M Linck; Susan J Hayflick; Don S Lin; Kevin P Battaile; S Ginat; Terry Burlingame; K Michael Gibson; M Honda; Akira Honda; G Salen; Stephen G Tint; William E Connor; Robert D Steiner
Fetal demise with Smith-Lemli-Opitz syndrome confirmed by tissue sterol analysis and the absence of measurable 7-dehydrocholesterol Delta(7)-reductase activity in chorionic villi.
Prenatal diagnosis 2000;20(3):238-40.
2000: Akira Honda; Gerald Salen; Sarah Shefer; Yasushi Matsuzaki; G Xu; Ashok K Batta; Stephen G Tint; Naomi Tanaka
Regulation of 25- and 27-hydroxylation side chain cleavage pathways for cholic acid biosynthesis in humans, rabbits, and mice. Assay of enzyme activities by high-resolution gas chromatography;-mass spectrometry.
Journal of lipid research 2000;41(3):442-51.
2000: Hongwei Yu; Stephen G Tint; Gerald Salen; Shailendra Patel
Detection of a common mutation in the RSH or Smith-Lemli-Opitz syndrome by a PCR-RFLP assay: IVS8-G-->C is found in over sixty percent of US propositi.
American journal of medical genetics 2000;90(4):347-50.
2000: Akira Honda; Gerald Salen; M Honda; Ashok K Batta; Stephen G Tint; G Xu; T S Chen; Naomi Tanaka; Sarah Shefer
3-Hydroxy-3-methylglutaryl-coenzyme A reductase activity is inhibited by cholesterol and up-regulated by sitosterol in sitosterolemic fibroblasts.
The Journal of laboratory and clinical medicine 2000;135(2):174-9.
2000: Guorong Xu; Benjamin L Shneider; Sarah Shefer; Lien B Nguyen; Ashok K Batta; Stephen G Tint; M Arrese; S Thevananther; L Ma; Siegfried Stengelin; Werner Kramer; D Greenblatt; M Pcolinsky; Gerald Salen
Ileal bile acid transport regulates bile acid pool, synthesis, and plasma cholesterol levels differently in cholesterol-fed rats and rabbits.
Journal of lipid research 2000;41(2):298-304.
1999: Lien B Nguyen; Guorong Xu; Sarah Shefer; Stephen G Tint; Ashok K Batta; Gerald Salen
Comparative regulation of hepatic sterol 27-hydroxylase and cholesterol 7alpha-hydroxylase activities in the rat, guinea pig, and rabbit: effects of cholesterol and bile acids.
Metabolism: clinical and experimental 1999;48(12):1542-8.
1999: Ashok K Batta; S C Datta; Stephen G Tint; D S Alberts; D L Earnest; Gerald Salen
A convenient synthesis of dinorbile acids: oxidative hydrolysis of norbile acid nitriles.
Steroids 1999;64(11):780-4.
1999: Akira Honda; Gerald Salen; Sarah Shefer; Ashok K Batta; M Honda; Guorong Xu; Stephen G Tint; Y Matsuzaki; J Shoda; N Tanaka
Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7alpha-hydroxylase and 27-hydroxylase activities in rat liver.
Journal of lipid research 1999;40(8):1520-8.
1999: Mira Irons; JA Nores; T L Stewart; Sabrina D Craigo; Diana Bianchi; M E D'Alton; Stephen G Tint; G Salen; L A Bradley
Antenatal therapy of Smith-Lemli-Opitz syndrome.
Fetal diagnosis and therapy 1999;14(3):133-7.
1999: G Xu; G Salen; Stephen G Tint; A K Batta; S Shefer
Campestanol (24-methyl-5alpha-cholestan-3beta-ol) absorption and distribution in New Zealand White rabbits: effect of dietary sitostanol.
Metabolism: clinical and experimental 1999;48(3):363-8.
1999: Linda A Bradley; Glenn E Palomaki; George J Knight; James E Haddow; J M Opitz; Mira Irons; Richard I Kelley; Stephen G Tint
Levels of unconjugated estriol and other maternal serum markers in pregnancies with Smith-Lemli-Opitz (RSH) syndrome fetuses.
American journal of medical genetics 1999;82(4):355-8.
1999: Guorong Xu; Gerald Salen; Sarah Shefer; Stephen G Tint; Lien B Nguyen; TS Chen; D Greenblatt
Increasing dietary cholesterol induces different regulation of classic and alternative bile acid synthesis.
The Journal of clinical investigation 1999;103(1):89-95.
1998: Brad Angle; Stephen G Tint; O A Yacoub; AL Clark
Atypical case of Smith-Lemli-Opitz syndrome: implications for diagnosis.
American journal of medical genetics 1998;80(4):322-6.
1998: Stephen G Tint; P Pentchev; G Xu; A K Batta; S Shefer; G Salen; Akira Honda
Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann-Pick type C phenotype.
Journal of inherited metabolic disease 1998;21(8):853-63.
1998: Sarah Shefer; Gerald Salen; Akira Honda; Ashok K Batta; Lien B Nguyen; Stephen G Tint; Y A Ioannou; R Desnick
Regulation of rat hepatic 3beta-hydroxysterol delta7-reductase: substrate specificity, competitive and non-competitive inhibition, and phosphorylation/dephosphorylation.
Journal of lipid research 1998;39(12):2471-6.
1998: M Honda; Stephen G Tint; Sarah Shefer; Akira Honda; Ashok K Batta; Guorong Xu; TS Chen; Gerald Salen
Accurate detection of Smith-Lemli-Opitz syndrome carriers by measurement of the rate of reduction of the ergosterol C-7 double bond in cultured skin fibroblasts.
Journal of inherited metabolic disease 1998;21(7):761-8.
1998: Ashok K Batta; Stephen G Tint; Guorong Xu; Sarah Shefer; Gerald Salen
Synthesis and intestinal metabolism of ursodeoxycholic acid conjugate with an antiinflammatory agent, 5-aminosalicylic acid.
Journal of lipid research 1998;39(8):1641-6.
1998: Guorong Xu; Gerald Salen; Sarah Shefer; Stephen G Tint; Lien B Nguyen; T T Parker; TS Chen; J Roberts; X Kong; D Greenblatt
Regulation of classic and alternative bile acid synthesis in hypercholesterolemic rabbits: effects of cholesterol feeding and bile acid depletion.
Journal of lipid research 1998;39(8):1608-15.
1998: Stephen G Tint; DN Abuelo; M Till; Martine Cordier; AK Batta; S Shefer; Akira Honda; M Honda; Guorong Xu; Mira Irons; Ellen R Elias; Gerald Salen
Fetal Smith-Lemli-Opitz syndrome can be detected accurately and reliably by measuring amniotic fluid dehydrocholesterols.
Prenatal diagnosis 1998;18(7):651-8.
1998: Guorong Xu; RJ Servatius; S Shefer; Stephen G Tint; WT O'Brien; AK Batta; Gerald Salen
Relationship between abnormal cholesterol synthesis and retarded learning in rats.
Metabolism: clinical and experimental 1998;47(7):878-82.
1998: Mira Irons; Stephen G Tint
Prenatal diagnosis of Smith-Lemli-Opitz syndrome.
Prenatal diagnosis 1998;18(4):369-72.
1998: M Honda; Stephen G Tint; Akira Honda; Lien B Nguyen; TS Chen; Sarah Shefer
7-Dehydrocholesterol down-regulates cholesterol biosynthesis in cultured Smith-Lemli-Opitz syndrome skin fibroblasts.
Journal of lipid research 1998;39(3):647-57.
1998: Masato Tsukahara; K Fujisawa; K Yamamoto; Masaki Hasui; Chikako Saito; T Yamamaka; Akira Honda; M Honda; Stephen G Tint; G Salen; J M Opitz
Smith-Lemli-Opitz syndrome in Japan.
American journal of medical genetics 1998;75(1):118-9.
1998: Akira Honda; Gerald Salen; Lien B Nguyen; Stephen G Tint; Ashok K Batta; Sarah Shefer
Down-regulation of cholesterol biosynthesis in sitosterolemia: diminished activities of acetoacetyl-CoA thiolase, 3-hydroxy-3-methylglutaryl-CoA synthase, reductase, squalene synthase, and 7-dehydrocholesterol delta7-reductase in liver and mononuclear leukocytes.
Journal of lipid research 1998;39(1):44-50.
1998: Lien B Nguyen; Sarah Shefer; Gerald Salen; Stephen G Tint; Ashok K Batta
Competitive inhibition of hepatic sterol 27-hydroxylase by sitosterol: decreased activity in sitosterolemia.
Proceedings of the Association of American Physicians 1998;110(1):32-9.
1998: Akira Honda; Gerald Salen; Lien B Nguyen; Guorong Xu; Stephen G Tint; Ashok K Batta; Sarah Shefer
Regulation of early cholesterol biosynthesis in rat liver: effects of sterols, bile acids, lovastatin, and BM 15.766 on 3-hydroxy-3-methylglutaryl coenzyme A synthase and acetoacetyl coenzyme A thiolase activities.
Hepatology (Baltimore, Md.) 1998;27(1):154-9.
1997: G Xu; Ruth M Elsey; Valentine A Lance; Bruce R. Javors; T S Chen; G Salen; Stephen G Tint
A study on biliary ductal system and bile fistula in the American alligator, Alligator mississippiensis.
The Journal of experimental zoology 1997;279(6):554-61.
1997: M M Cobb; Gerald Salen; Stephen G Tint
Comparative effect of dietary sitosterol on plasma sterols and cholesterol and bile acid synthesis in a sitosterolemic homozygote and heterozygote subject.
Journal of the American College of Nutrition 1997;16(6):605-13.
1997: Guorong Xu; Gerald Salen; Sarah Shefer; Stephen G Tint; Betsy T Kren; Lien B Nguyen; Clifford J Steer; TS Chen; L Salen; D Greenblatt
Increased bile acid pool inhibits cholesterol 7 alpha-hydroxylase in cholesterol-fed rabbits.
Gastroenterology 1997;113(6):1958-65.
1997: L Lanoue; Deborah B Dehart; Myron E Hinsdale; Nobuyo Maeda; Stephen G Tint; Kathleen K Sulik
Limb, genital, CNS, and facial malformations result from gene/environment-induced cholesterol deficiency: further evidence for a link to sonic hedgehog.
American journal of medical genetics 1997;73(1):24-31.
1997: Ashok K Batta; Gerald Salen; Stephen G Tint; Akira Honda; Sarah Shefer
Synthesis of [3 alpha-3H]7-dehydrocholesterol via stable tritiated 4-phenyl-1,2,4-triazoline-3,5-dione derivative.
Steroids 1997;62(11):700-2.
1997: Sarah Shefer; Gerald Salen; Akira Honda; Ashok K Batta; S Hauser; Stephen G Tint; M Honda; T Chen; M F Holick; Lien B Nguyen
Rapid identification of Smith-Lemli-Opitz syndrome homozygotes and heterozygotes (carriers) by measurement of deficient 7-dehydrocholesterol-delta 7-reductase activity in fibroblasts.
Metabolism: clinical and experimental 1997;46(7):844-50.
1997: J A Canick; DN Abuelo; Linda A Bradley; Stephen G Tint
Maternal serum marker levels in two pregnancies affected with Smith-Lemli-Opitz syndrome.
Prenatal diagnosis 1997;17(2):187-9.
1997: Deborah B Dehart; L Lanoue; Stephen G Tint; Kathleen K Sulik
Pathogenesis of malformations in a rodent model for Smith-Lemli-Opitz syndrome.
American journal of medical genetics 1997;68(3):328-37.
1997: Mira Irons; Ellen R Elias; DN Abuelo; M J Bull; Carol L Greene; V P Johnson; Laura Davis Keppen; C Schanen; Stephen G Tint; Gerald Salen
Treatment of Smith-Lemli-Opitz syndrome: results of a multicenter trial.
American journal of medical genetics 1997;68(3):311-4.
1997: Ellen R Elias; Mira Irons; A D Hurley; Stephen G Tint; Gerald Salen
Clinical effects of cholesterol supplementation in six patients with the Smith-Lemli-Opitz syndrome (SLOS)
American journal of medical genetics 1997;68(3):305-10.
1997: Akira Honda; Ashok K Batta; Gerald Salen; Stephen G Tint; TS Chen; Sarah Shefer
Screening for abnormal cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome: rapid determination of plasma 7-dehydrocholesterol by ultraviolet spectrometry.
American journal of medical genetics 1997;68(3):288-93.
1997: Akira Honda; Stephen G Tint; G Salen; Richard I Kelley; M Honda; A K Batta; T S Chen; S Shefer
Sterol concentrations in cultured Smith-Lemli-Opitz syndrome skin fibroblasts: diagnosis of a biochemically atypical case of the syndrome.
American journal of medical genetics 1997;68(3):282-7.
1997: Richard M Pauli; Marc S Williams; KD Josephson; Stephen G Tint
Smith-Lemli-Opitz syndrome: thirty-year follow-up of "S" of "RSH" syndrome.
American journal of medical genetics 1997;68(3):260-2.
1997: Gerald Salen; Sarah Shefer; L Nguyen; G C Ness; Stephen G Tint; Ashok K Batta
Sitosterolemia.
Sub-cellular biochemistry 1997;28():453-76.
1997: Stephen G Tint; AK Batta; Guorong Xu; S Shefer; A Honda; Mira Irons; Ellen R Elias; Gerald Salen
The Smith-Lemli-Opitz syndrome: a potentially fatal birth defect caused by a block in the last enzymatic step in cholesterol biosynthesis.
Sub-cellular biochemistry 1997;28():117-44.
1996: M Honda; Stephen G Tint; Akira Honda; A K Batta; T S Chen; S Shefer; G Salen
Measurement of 3 beta-hydroxysteroid delta 7-reductase activity in cultured skin fibroblasts utilizing ergosterol as a substrate: a new method for the diagnosis of the Smith-Lemli-Opitz syndrome.
Journal of lipid research 1996;37(11):2433-8.
1996: G Xu; G Salen; Sarah Shefer; G C Ness; Lien B Nguyen; Stephen G Tint; T S Parker; J Roberts; A K Batta; TS Chen; Z Zhao; X Kong
Increasing hepatic cholesterol 7alpha-hydroxylase reduces plasma cholesterol concentrations in normocholesterolemic and hypercholesterolemic rabbits.
Hepatology (Baltimore, Md.) 1996;24(4):882-7.
1996: Akira Honda; Stephen G Tint; Sarah Shefer; A K Batta; M Honda; G Salen
Effect of YM 9429, a potent teratogen, on cholesterol biosynthesis in cultured cells and rat liver microsomes.
Steroids 1996;61(9):544-8.
1996: G Xu; G Salen; M Lea; Stephen G Tint; L B Nguyen; A K Batta; T S Chen; S Shefer
Blocking late cholesterol biosynthesis inhibits the growth of transplanted Morris hepatomas (7288CTC) in rats.
Hepatology (Baltimore, Md.) 1996;24(2):440-5.
1996: Akira Honda; Sarah Shefer; G Salen; G Xu; A K Batta; Stephen G Tint; M Honda; T C Chen; M F Holick
Regulation of the last two enzymatic reactions in cholesterol biosynthesis in rats: effects of BM 15.766, cholesterol, cholic acid, lovastatin, and their combinations.
Hepatology (Baltimore, Md.) 1996;24(2):435-9.
1996: G Salen; Sarah Shefer; A K Batta; Stephen G Tint; G Xu; Akira Honda; Mira Irons; Ellen R Elias
Abnormal cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome.
Journal of lipid research 1996;37(6):1169-80.
1996: M M Cobb; G Salen; Stephen G Tint; J Greenspan; L B Nguyen
Sitosterolemia: opposing effects of cholestyramine and lovastatin on plasma sterol levels in a homozygous girl and her heterozygous father.
Metabolism: clinical and experimental 1996;45(6):673-9.
1996: G Salen; S Shefer; A K Batta; Stephen G Tint; G Xu; Akira Honda
Abnormal cholesterol biosynthesis in sitosterolaemia and the Smith-Lemli-Opitz syndrome.
Journal of inherited metabolic disease 1996;19(4):391-400.
1995: G Salen; Stephen G Tint; G Xu; A K Batta; Mira Irons; Ellen R Elias
Abnormal cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome.
The Italian journal of gastroenterology 1995;27(9):506-8.
1995: A K Batta; G Salen; Stephen G Tint; Sarah Shefer
Identification of 19-nor-5,7,9(10)-cholestatrien-3 beta-ol in patients with Smith-Lemli-Opitz syndrome.
Journal of lipid research 1995;36(11):2413-8.
1995: Sarah Shefer; G Salen; A K Batta; Akira Honda; Stephen G Tint; Mira Irons; Ellen R Elias; T C Chen; M F Holick
Markedly inhibited 7-dehydrocholesterol-delta 7-reductase activity in liver microsomes from Smith-Lemli-Opitz homozygotes.
The Journal of clinical investigation 1995;96(4):1779-85.
1995: Sarah Shefer; Betsy T Kren; G Salen; Clifford J Steer; Lien B Nguyen; T Chen; Stephen G Tint; A K Batta
Regulation of bile acid synthesis by deoxycholic acid in the rat: different effects on cholesterol 7 alpha-hydroxylase and sterol 27-hydroxylase.
Hepatology (Baltimore, Md.) 1995;22(4 Pt 1):1215-21.
1995: G Xu; G Salen; Sarah Shefer; G C Ness; TS Chen; Z Zhao; L Salen; Stephen G Tint
Treatment of the cholesterol biosynthetic defect in Smith-Lemli-Opitz syndrome reproduced in rats by BM 15.766.
Gastroenterology 1995;109(4):1301-7.
1995: C P Martucci; D G Miller; B Levine; Stephen G Tint; J Fishman
Changes in serum bile acids in normal human subjects following the adoption of a low-fat diet.
Annals of the New York Academy of Sciences 1995;768():331-3.
1995: Stephen G Tint; G Salen; A K Batta; Sarah Shefer; Mira Irons; Ellen R Elias; DN Abuelo; V P Johnson; M Lambert; R Lutz
Correlation of severity and outcome with plasma sterol levels in variants of the Smith-Lemli-Opitz syndrome.
The Journal of pediatrics 1995;127(1):82-7.
1995: Akira Honda; Stephen G Tint; G Salen; A K Batta; T S Chen; S Shefer
Defective conversion of 7-dehydrocholesterol to cholesterol in cultured skin fibroblasts from Smith-Lemli-Opitz syndrome homozygotes.
Journal of lipid research 1995;36(7):1595-601.
1995: A K Batta; S K Aggarwal; Stephen G Tint; M Batta; G Salen
Capillary gas-liquid chromatography of 6-hydroxylated bile acids.
Journal of chromatography. A 1995;704(1):228-33.
1995: T L Alley; Brian A Gray; S H Lee; S W Scherer; L C Tsui; Stephen G Tint; Charles A Williams; Roberto T Zori; Margaret R Wallace
Identification of a yeast artificial chromosome clone spanning a translocation breakpoint at 7q32.1 in a Smith-Lemli-Opitz syndrome patient.
American journal of human genetics 1995;56(6):1411-6.
1995: Dianne Abuelo; Stephen G Tint; Richard I Kelley; A K Batta; S Shefer; G Salen
Prenatal detection of the cholesterol biosynthetic defect in the Smith-Lemli-Opitz syndrome by the analysis of amniotic fluid sterols.
American journal of medical genetics 1995;56(3):281-5.
1995: G Pierquin; P Peeters; F Roels; Esther Vamos; J M Brucher; Stephen G Tint; Akira Honda; Nicole Van Regemorter
Severe Smith-Lemli-Opitz syndrome with prolonged survival and lipid abnormalities.
American journal of medical genetics 1995;56(3):276-80.
1995: MJ Seller; J Russell; Stephen G Tint
Unusual case of Smith-Lemli-Opitz syndrome "type II".
American journal of medical genetics 1995;56(3):265-8.
1995: G Xu; G Salen; Sarah Shefer; G C Ness; Lien B Nguyen; T S Parker; TS Chen; Z Zhao; T M Donnelly; Stephen G Tint
Unexpected inhibition of cholesterol 7 alpha-hydroxylase by cholesterol in New Zealand white and Watanabe heritable hyperlipidemic rabbits.
The Journal of clinical investigation 1995;95(4):1497-504.
1995: A K Batta; Stephen G Tint; Sarah Shefer; D Abuelo; G Salen
Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3 beta-ol) in patients with Smith-Lemli-Opitz syndrome.
Journal of lipid research 1995;36(4):705-13.
1995: G Xu; G Salen; Sarah Shefer; G C Ness; TS Chen; Z Zhao; Stephen G Tint
Reproducing abnormal cholesterol biosynthesis as seen in the Smith-Lemli-Opitz syndrome by inhibiting the conversion of 7-dehydrocholesterol to cholesterol in rats.
The Journal of clinical investigation 1995;95(1):76-81.
1995: Stephen G Tint; M Seller; R Hughes-Benzie; A K Batta; S Shefer; D Genest; M Irons; E Elias; G Salen
Markedly increased tissue concentrations of 7-dehydrocholesterol combined with low levels of cholesterol are characteristic of the Smith-Lemli-Opitz syndrome.
Journal of lipid research 1995;36(1):89-95.
1994: G Salen; A K Batta; Stephen G Tint; S Shefer; G C Ness
Inverse relationship between plasma cholestanol concentrations and bile acid synthesis in sitosterolemia.
Journal of lipid research 1994;35(10):1878-87.
1994: G Salen; A K Batta; Stephen G Tint; S Shefer
Comparative effects of lovastatin and chenodeoxycholic acid on plasma cholestanol levels and abnormal bile acid metabolism in cerebrotendinous xanthomatosis.
Metabolism: clinical and experimental 1994;43(8):1018-22.
1994: Lien B Nguyen; G Salen; Sarah Shefer; J Bullock; T Chen; Stephen G Tint; I R Chowdhary; S Lerner
Deficient ileal 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in sitosterolemia: sitosterol is not a feedback inhibitor of intestinal cholesterol biosynthesis.
Metabolism: clinical and experimental 1994;43(7):855-9.
1994: Mira Irons; Ellen R Elias; Stephen G Tint; G Salen; R Frieden; T M Buie; M Ampola
Abnormal cholesterol metabolism in the Smith-Lemli-Opitz syndrome: report of clinical and biochemical findings in four patients and treatment in one patient.
American journal of medical genetics 1994;50(4):347-52.
1994: Stephen G Tint; Mira Irons; Ellen R Elias; A K Batta; R Frieden; T S Chen; G Salen
Defective cholesterol biosynthesis associated with the Smith-Lemli-Opitz syndrome.
The New England journal of medicine 1994;330(2):107-13.
1993: A Chawla; Ellen Kahn; J Becker; H Cohen; Stephen G Tint; S Shefer; S E Fisher
Focal nodular hyperplasia of the liver and hypercholesterolemia in a child with VACTERL syndrome.
Journal of pediatric gastroenterology and nutrition 1993;17(4):434-7.
1993: G Xu; G Salen; Sarah Shefer; A K Batta; G C Ness; Lien B Nguyen; Z Zhao; TS Chen; W Niemann; Stephen G Tint
Different feedback regulation of hepatic cholesterol and bile acid synthesis by glycodeoxycholic acid in rabbits.
Gastroenterology 1993;105(4):1192-9.
1993: Stephen G Tint
Cholesterol defect in Smith-Lemli-Opitz syndrome.
American journal of medical genetics 1993;47(4):573-4.
1993: Mira Irons; Ellen R Elias; G Salen; Stephen G Tint; A K Batta
Defective cholesterol biosynthesis in Smith-Lemli-Opitz syndrome.
Lancet 1993;341(8857):1414.
1993: A K Batta; G Salen; R Mirchandani; Stephen G Tint; Sarah Shefer; M Batta; J Abroon; Christopher B O'Brien; J R Senior
Effect of long-term treatment with ursodiol on clinical and biochemical features and biliary bile acid metabolism in patients with primary biliary cirrhosis.
The American journal of gastroenterology 1993;88(5):691-700.
1993: Bishambar Dayal; G Salen; J Padia; Sarah Shefer; Stephen G Tint; G Sasso; T H Williams
Bile alcohol glucuronides: regioselective O-glucuronidation of 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol and 24-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol.
Carbohydrate research 1993;240():133-42.
1993: V M Berginer; S Shany; D Alkalay; J Berginer; S Dekel; G Salen; Stephen G Tint; D Gazit
Osteoporosis and increased bone fractures in cerebrotendinous xanthomatosis.
Metabolism: clinical and experimental 1993;42(1):69-74.
1992: Sarah Shefer; Lien B Nguyen; G Salen; G C Ness; I R Chowdhary; S Lerner; A K Batta; Stephen G Tint
Differing effects of cholesterol and taurocholate on steady state hepatic HMG-CoA reductase and cholesterol 7 alpha-hydroxylase activities and mRNA levels in the rat.
Journal of lipid research 1992;33(8):1193-200.
1992: G Salen; Sarah Shefer; L Nguyen; G C Ness; Stephen G Tint; V Shore
Sitosterolemia.
Journal of lipid research 1992;33(7):945-55.
1992: Stephen G Tint; H Dyrszka; B Sanghavi; G Patel; S Patel; S Shefer; G Salen
Lithotripsy plus ursodiol is superior to ursodiol alone for cholesterol gallstones.
Gastroenterology 1992;102(6):2042-9.
1992: G Salen; Stephen G Tint; S Shefer; V Shore; L Nguyen
Increased sitosterol absorption is offset by rapid elimination to prevent accumulation in heterozygotes with sitosterolemia.
Arteriosclerosis and thrombosis : a journal of vascular biology / American Heart Association 1992;12(5):563-8.
1992: G Xu; G Salen; A K Batta; Sarah Shefer; Lien B Nguyen; W Niemann; TS Chen; R Arora-Mirchandani; G C Ness; Stephen G Tint
Glycocholic acid and glycodeoxycholic acid but not glycoursocholic acid inhibit bile acid synthesis in the rabbit.
Gastroenterology 1992;102(5):1717-23.
1992: Bishambar Dayal; G Salen; J Padia; Sarah Shefer; Stephen G Tint; T H Williams; V Toome; G Sasso
Stereoselective synthesis of (24R and 24S) 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25-pentols and (25R and 25S) 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25,26-pentols using a modified osmium-catalyzed Sharpless asymmetric dihydroxylation process.
Chemistry and physics of lipids 1992;61(3):271-81.
1992: Stephen G Tint; A K Batta; Bishambar Dayal; N Kovell; S Shefer; G Salen
Metabolism of ursocholic acid in humans: conversion of ursocholic acid to deoxycholic acid.
Hepatology (Baltimore, Md.) 1992;15(4):645-50.
1992: Alan F Hofmann; J Sjövall; G Kurz; A Radominska; Claudio D Schteingart; Stephen G Tint; Z Reno Vlahcevic; K D Setchell
A proposed nomenclature for bile acids.
Journal of lipid research 1992;33(4):599-604.
1991: Lien B Nguyen; M Cobb; Sarah Shefer; G Salen; G C Ness; Stephen G Tint
Regulation of cholesterol biosynthesis in sitosterolemia: effects of lovastatin, cholestyramine, and dietary sterol restriction.
Journal of lipid research 1991;32(12):1941-8.
1991: G Salen; Stephen G Tint; Sarah Shefer
Treatment of cholesterol gallstones with litholytic bile acids.
Gastroenterology clinics of North America 1991;20(1):171-82.
1991: Sarah Shefer; Lien B Nguyen; G Salen; G C Ness; Stephen G Tint; A K Batta; S Hauser; I Rani
Regulation of cholesterol 7 alpha-hydroxylase by hepatic 7 alpha-hydroxylated bile acid flux and newly synthesized cholesterol supply.
The Journal of biological chemistry 1991;266(5):2693-6.
1990: Peter F Belamarich; Richard J Deckelbaum; Thomas J Starc; B E Dobrin; Stephen G Tint; G Salen
Response to diet and cholestyramine in a patient with sitosterolemia.
Pediatrics 1990;86(6):977-81.
1990: Lien B Nguyen; Sarah Shefer; G Salen; G C Ness; Stephen G Tint; F G Zaki; I Rani
A molecular defect in hepatic cholesterol biosynthesis in sitosterolemia with xanthomatosis.
The Journal of clinical investigation 1990;86(3):923-31.
1990: G Salen; Stephen G Tint; S Shefer
Oral dissolution treatment of gallstones with bile acids.
Seminars in liver disease 1990;10(3):181-6.
1990: Stephen G Tint; G R Xu; A K Batta; Sarah Shefer; W Niemann; G Salen
Ursodeoxycholic acid, chenodeoxycholic acid, and 7-ketolithocholic acid are primary bile acids of the guinea pig.
Journal of lipid research 1990;31(7):1301-6.
1990: Bishambar Dayal; G Salen; B Toome; Stephen G Tint; Sarah Shefer; J Padia
Lithium hydroxide/aqueous methanol: mild reagent for the hydrolysis of bile acid methyl esters.
Steroids 1990;55(5):233-7.
1990: Lien B Nguyen; G Salen; Sarah Shefer; Stephen G Tint; V Shore; G C Ness
Decreased cholesterol biosynthesis in sitosterolemia with xanthomatosis: diminished mononuclear leukocyte 3-hydroxy-3-methylglutaryl coenzyme A reductase activity and enzyme protein associated with increased low-density lipoprotein receptor function.
Metabolism: clinical and experimental 1990;39(4):436-43.
1990: Sarah Shefer; L Nguyen; G Salen; A K Batta; D Brooker; F G Zaki; I Rani; Stephen G Tint
Feedback regulation of bile-acid synthesis in the rat. Differing effects of taurocholate and tauroursocholate.
The Journal of clinical investigation 1990;85(4):1191-8.
1990: L Nguyen; G Salen; Sarah Shefer; V Shore; Stephen G Tint; G Ness
Unexpected failure of bile acid malabsorption to stimulate cholesterol synthesis in sitosterolemia with xanthomatosis. Comparison with lovastatin.
Arteriosclerosis (Dallas, Tex.) 1990;10(2):289-97.
1990: Bishambar Dayal; G Salen; Stephen G Tint; Sarah Shefer; S W Benz
Use of positive ion fast atom bombardment mass spectrometry for rapid identification of a bile alcohol glucuronide isolated from cerebrotendinous xanthomatosis patients.
Steroids 1990;55(2):74-8.
1989: A K Batta; R Arora; G Salen; Stephen G Tint; D Eskreis; S Katz
Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment.
Journal of lipid research 1989;30(12):1953-62.
1989: A K Batta; G Salen; R Arora; Sarah Shefer; Stephen G Tint; J Abroon; D Eskreis; S Katz
Effect of ursodeoxycholic acid on bile acid metabolism in primary biliary cirrhosis.
Hepatology (Baltimore, Md.) 1989;10(4):414-9.
1989: G Salen; V Shore; Stephen G Tint; T Forte; Sarah Shefer; I Horak; E Horak; Bishambar Dayal; L Nguyen; A K Batta
Increased sitosterol absorption, decreased removal, and expanded body pools compensate for reduced cholesterol synthesis in sitosterolemia with xanthomatosis.
Journal of lipid research 1989;30(9):1319-30.
1989: Stephen G Tint; Henry N Ginsberg; G Salen; N A Le; Sarah Shefer
Chenodeoxycholic acid normalizes elevated lipoprotein secretion and catabolism in cerebrotendinous xanthomatosis.
Journal of lipid research 1989;30(5):633-40.
1989: G Salen; Stephen G Tint
Nonsurgical treatment of gallstones.
The New England journal of medicine 1989;320(10):665-6.
1988: Sarah Shefer; G Salen; L Nguyen; A K Batta; V Packin; Stephen G Tint; S Hauser
Competitive inhibition of bile acid synthesis by endogenous cholestanol and sitosterol in sitosterolemia with xanthomatosis. Effect on cholesterol 7 alpha-hydroxylase.
The Journal of clinical investigation 1988;82(6):1833-9.
1988: Lien B Nguyen; Sarah Shefer; G Salen; I Horak; Stephen G Tint; D J McNamara
The effect of abnormal plasma and cellular sterol content and composition on low density lipoprotein uptake and degradation by monocytes and lymphocytes in sitosterolemia with xanthomatosis.
Metabolism: clinical and experimental 1988;37(4):346-51.
1987: A K Batta; G Salen; Sarah Shefer; Stephen G Tint; M Batta
Increased plasma bile alcohol glucuronides in patients with cerebrotendinous xanthomatosis: effect of chenodeoxycholic acid.
Journal of lipid research 1987;28(8):1006-12.
1987: G Salen; V Berginer; V Shore; I Horak; E Horak; Stephen G Tint; Sarah Shefer
Increased concentrations of cholestanol and apolipoprotein B in the cerebrospinal fluid of patients with cerebrotendinous xanthomatosis. Effect of chenodeoxycholic acid.
The New England journal of medicine 1987;316(20):1233-8.
1986: Bishambar Dayal; G Salen; V Toome; Stephen G Tint
Configuration at C-25 in 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25,26-pentol excreted by patients with cerebrotendinous xanthomatosis: circular dichroism and 13C-NMR studies.
Journal of lipid research 1986;27(12):1328-32.
1986: Stephen G Tint; G Salen; S Shefer
Effect of ursodeoxycholic acid and chenodeoxycholic acid on cholesterol and bile acid metabolism.
Gastroenterology 1986;91(4):1007-18.
1986: Stephen G Tint; J Bullock; A K Batta; Sarah Shefer; G Salen
Ursodeoxycholic acid, 7-ketolithocholic acid, and chenodeoxycholic acid are primary bile acids of the nutria (Myocastor coypus).
Gastroenterology 1986;90(3):702-9.
1985: A B Stefaniwsky; Stephen G Tint; J Speck; S Shefer; G Salen
Ursodeoxycholic acid treatment of bile reflux gastritis.
Gastroenterology 1985;89(5):1000-4.
1985: G Salen; I Horak; M Rothkopf; J L Cohen; J Speck; Stephen G Tint; V Shore; Bishambar Dayal; T Chen; Sarah Shefer
Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis.
Journal of lipid research 1985;26(9):1126-33.
1985: G Salen; S Shefer; Stephen G Tint; G Nicolau; Bishambar Dayal; A K Batta
Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26.
The Journal of clinical investigation 1985;76(2):744-51.
1985: Bishambar Dayal; Stephen G Tint; V Toome; A K Batta; Sarah Shefer; G Salen
Synthesis and structure of 26 (or 27)-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24S,25 xi-pentol isolated from the urine and feces of a patient with sitosterolemia and xanthomatosis.
Journal of lipid research 1985;26(3):298-305.
1985: G Salen; Peter O Kwiterovich; Sarah Shefer; Stephen G Tint; I Horak; V Shore; Bishambar Dayal; E Horak
Increased plasma cholestanol and 5 alpha-saturated plant sterol derivatives in subjects with sitosterolemia and xanthomatosis.
Journal of lipid research 1985;26(2):203-9.
1984: G Salen; S Shefer; Stephen G Tint
Transformation of 4-cholesten-3-one and 7 alpha-hydroxy-4-cholesten-3-one into cholestanol and bile acids in cerebrotendinous xanthomatosis.
Gastroenterology 1984;87(2):276-83.
1984: Bishambar Dayal; G Salen; Stephen G Tint; A K Batta; S Shefer
Synthesis of the putative metabolites of plant sterols: (24R)- and (24S)-24-methyl-5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrols and 24-ethyl-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24 xi-tetrol.
Journal of lipid research 1984;25(8):865-70.
1984: Bishambar Dayal; D N Greeley; T H Williams; Stephen G Tint; G Salen
Stereospecific synthesis of 3 beta-hydroxylated bile alcohols.
Journal of lipid research 1984;25(6):646-50.
1983: Bishambar Dayal; G Salen; Stephen G Tint; C Biswas
A facile synthesis of [3 alpha-3 H]beta -sitosterol.
Steroids 1983;42(6):635-40.
1983: R Fleischmajer; E J Schaefer; A E Gal; P G Pentchev; Stephen G Tint
Normolipemic subcutaneous xanthomatosis.
The American journal of medicine 1983;75(6):1065-70.
1983: Bishambar Dayal; Stephen G Tint; D N Greeley; T H Williams; G Salen
Identification of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25, 26-pentol in cerebrotendinous xanthomatosis.
Steroids 1983;42(4):441-8.
1983: Bishambar Dayal; Stephen G Tint; A K Batta; Sarah Shefer; G Salen; A K Bose; B N Pramanik
Synthesis of 3 alpha,7 alpha,12 alpha,25-tetrahydroxy-5 beta-cholestan-24-one, an intermediate in the 25-hydroxylation pathway of cholic acid biosynthesis from cholesterol.
Journal of lipid research 1983;24(2):208-10.
1983: A K Batta; G Salen; S Shefer; Bishambar Dayal; Stephen G Tint
Configuration at C-25 in 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestan-26-oic acid isolated from human bile.
Journal of lipid research 1983;24(1):94-6.
1982: A K Batta; G Salen; S Shefer; Stephen G Tint; Bishambar Dayal
The effect of tauroursodeoxycholic acid and taurine supplementation on biliary bile acid composition.
Hepatology (Baltimore, Md.) 1982;2(6):811-6.
1982: Stephen G Tint; G Salen; A Colalillo; D Graber; D Verga; J Speck; S Shefer
Ursodeoxycholic acid: a safe and effective agent for dissolving cholesterol gallstones.
Annals of internal medicine 1982;97(3):351-6.
1982: G Salen; D Verga; A K Batta; Stephen G Tint; S Shefer
Effect of 7-ketolithocholic acid on bile acid metabolism in humans.
Gastroenterology 1982;83(2):341-7.
1982: Bishambar Dayal; Stephen G Tint; A K Batta; J Speck; A K Khachadurian; Sarah Shefer; G Salen
Identification of 5 alpha-stanols in patients with sitosterolemia and xanthomatosis: stereochemistry of the protonolysis of steroidal organoboranes.
Steroids 1982;40(2):233-43.
1982: A K Batta; Stephen G Tint; Bishambar Dayal; S Shefer; G Salen
Improved synthesis of 3 alpha, 7 alpha, 12 alpha, 24 = xi-tetrahydroxy-5 beta-cholestan-26-oic acid.
Steroids 1982;39(6):693-702.
1982: Stephen G Tint; G Salen
Biosynthesis of cholesterol, lanosterol, and delta 7-cholestenol, but not cholestanol, in cultured fibroblasts from normal individuals and patients with cerebrotendinous xanthomatosis.
Journal of lipid research 1982;23(4):597-603.
1982: Sarah Shefer; G Salen; F W Cheng; Bishambar Dayal; A K Batta; Stephen G Tint; A K Bose; B N Pramanik
Chemical ionization-mass spectrometric approach to structure determination of an intermediate in bile acid biosynthesis.
Analytical biochemistry 1982;121(1):23-30.
1981: V Shore; G Salen; F W Cheng; T Forte; Sarah Shefer; Stephen G Tint; F T Lindgren
Abnormal high density lipoproteins in cerebrotendinous xanthomatosis.
The Journal of clinical investigation 1981;68(5):1295-304.
1981: R Fleischmajer; Stephen G Tint; H D Bennett
Normolipemic tendon and tuberous xanthomas.
Journal of the American Academy of Dermatology 1981;5(3):290-6.
1981: Stephen G Tint; Bishambar Dayal; A K Batta; S Shefer; T Joanen; L McNease; G Salen
The fecal bile acids and sterols of Alligator mississippiensis.
Gastroenterology 1981;81(1):114-9.
1981: Bishambar Dayal; J Speck; E Bagan; Stephen G Tint; G Salen
p-Toluenesulfonic acid/methanol: mild reagent for the preparation of bile acid methyl esters.
Steroids 1981;37(2):239-42.
1981: Bishambar Dayal; Stephen G Tint; A K Batta; S Shefer; G Salen
Synthesis of biological precursors of cholic acid II.
Steroids 1981;37(2):205-11.
1980: G Salen; A Colalillo; D Verga; E Bagan; Stephen G Tint; S Shefer
Effect of high and low doses of ursodeoxycholic acid on gallstone dissolution in humans.
Gastroenterology 1980;78(6):1412-8.
1980: Stephen G Tint; Bishambar Dayal; A K Batta; S Shefer; T Joanen; L McNease; G Salen
Biliary bile acids, bile alcohols, and sterols of Alligator mississippiensis.
Journal of lipid research 1980;21(1):110-7.
1979: Bishambar Dayal; Stephen G Tint; G Salen
C26-analogs of naturally occurring bile alcohols-II. Preparation of 24-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,23-tetrols (23R and 23S) and 24-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,26-tetrols (25R and 25S) by a hydroboration procedure.
Steroids 1979;34(5):581-8.
1979: T Fedorowski; G Salen; Stephen G Tint; E Mosbach
Transformation of chenodeoxycholic acid and ursodeoxycholic acid by human intestinal bacteria.
Gastroenterology 1979;77(5):1068-73.
1979: Bishambar Dayal; E Bagan; Stephen G Tint; S Shefer; G Salen
Preparation of [3beta-3H] labeled bile acids and bile alcohols.
Steroids 1979;34(3):259-72.
1979: A K Batta; G Salen; Stephen G Tint; S Shefer
Improved synthesis of 5 beta-cholestan-26-oic acids.
Steroids 1979;33(5):589-94.
1979: Bishambar Dayal; Stephen G Tint; S Shefer; G Salen
Configurational assignment of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 23, 25-pentol excreted by patients with cerebrotendinous xanthomatosis (a circular dichroism study).
Steroids 1979;33(3):327-38.
1979: G Salen; S Shefer; F W Cheng; Bishambar Dayal; A K Batta; Stephen G Tint
Cholic acid biosynthesis: the enzymatic defect in cerebrotendinous xanthomatosis.
The Journal of clinical investigation 1979;63(1):38-44.
1978: N Hoshita; S Shefer; F W Cheng; Bishambar Dayal; A K Batta; Stephen G Tint; G Salen; E H Mosbach
Biosynthesis of chenodeoxycholic acid: side-chain hydroxylation of 5 beta-cholestane-3 alpha, 7 alpha-diol by subcellular fractions of guinea pig liver.
Lipids 1978;13(12):961-5.
1978: Stephen G Tint; Bishambar Dayal; A K Batta; S Shefer; F W Cheng; G Salen; E H Mosbach
Gas-liquid chromatography-mass spectrometry of trimethylsilyl ethers of bile alcohols.
Journal of lipid research 1978;19(8):956-66.
1978: Bishambar Dayal; A K Batta; S Shefer; Stephen G Tint; G Salen
Synthesis of biological precursors of cholic acid.
Steroids 1978;32(3):337-44.
1978: S Shefer; F W Cheng; A K Batta; Bishambar Dayal; Stephen G Tint; G Salen; E H Mosbach
Stereospecific side chain hydroxylations in the biosynthesis of chenodeoxycholic acid.
The Journal of biological chemistry 1978;253(18):6386-92.
1978: S Shefer; F W Cheng; A K Batta; Bishambar Dayal; Stephen G Tint; G Salen
Biosynthesis of chenodeoxycholic acid in man: stereospecific side-chain hydroxylations of 5beta-cholestane-3alpha,7alpha-diol.
The Journal of clinical investigation 1978;62(3):539-45.
1978: Bishambar Dayal; A K Batta; S Shefer; Stephen G Tint; G Salen; E H Mosbach
Preparation of 24(R)- and 24(S)-5beta-cholestane-3alpha,7alpha,24-triols and 25(R)- and 25(S)-5beta-cholestane-3alpha,7alpha,26-triols by a hydroboration procedure.
Journal of lipid research 1978;19(2):191-6.
1978: Bishambar Dayal; G Salen; Stephen G Tint; V Toome; S Shefer; E H Mosbach
Absolute configuration of pentahydroxy bile alcohols excreted by patients with cerebrotendinous xanthomatosis: a circular dichroism study.
Journal of lipid research 1978;19(2):187-90.
1978: A K Batta; Bishambar Dayal; Stephen G Tint; S Shefer; V Toome; G Salen; E H Mosbach
Preparation and characterization of (24R and 24S)-5beta-cholestane-3alpha,7alpha,24,25-tetrols and (24R and 24S)-5beta-cholestane-3alpha,24,25-triols.
Steroids 1978;31(1):99-111.
1977: T Fedorowski; G Salen; A Calallilo; Stephen G Tint; E H Mosbach; J C Hall
Metabolism of ursodeoxycholic acid in man.
Gastroenterology 1977;73(5):1131-7.
1977: Stephen G Tint; G Salen
Evidence for the early reduction of the 24,25 double bond in the conversion of lanosterol to cholesterol in cerebrotendinous xanthomatosis.
Metabolism: clinical and experimental 1977;26(7):721-9.
1977: F W Cheng; S Shefer; Bishambar Dayal; Stephen G Tint; T Setoguchi; G Salen; E H Mosbach
Cholic acid biosynthesis: conversion of 5beta-cholestane-3alpha,7alpha,12alpha,25-tetrol into 5beta-cholestane-3alpha,7alpha, 12alpha,24beta,25-pentol by human and rat liver microsomes.
Journal of lipid research 1977;18(1):6-13.
1976: Bishambar Dayal; S Shefer; Stephen G Tint; G Salen; E H Mosbach
C26-Analogs of naturally occurring C27 bile alcohols.
Journal of lipid research 1976;17(5):478-84.
1976: S Shefer; F W Cheng; Bishambar Dayal; S Hauser; Stephen G Tint; G Salen; E H Mosbach
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
The Journal of clinical investigation 1976;57(4):897-903.
1976: Bishambar Dayal; S Shefer; Stephen G Tint; G Salen; E H Mosbach
Synthesis of 5beta-cholestane-3alpha, 7alpha, 12alpha, 25-tetrol and 5beta-cholestane-3alpha, 7alpha, 245, 25-pentol.
Journal of lipid research 1976;17(1):74-7.
1975: S Shefer; Bishambar Dayal; Stephen G Tint; G Salen; E H Mosbach
Identification of pentahydroxy bile alcohols in cerebrotendinous xanthomatosis: characterization of 5beta-cholestane-3alpha, 7alpha, 12alpha, 24xi, 25-pentol and 5beta-cholestane-3alpha, 7alpha, 12alpha, 23xi, 25-pentol.
Journal of lipid research 1975;16(4):280-6.
1975: Bertram Cohen; Stephen G Tint; T Kuramoto; E H Mosbach
New bile alcohols--synthesis of 5beta-cholestane-3alpha, 7alpha, 25-triol and 5beta-cholestane-3alpha, 7alpha, 25-24 (14C)-triol.
Steroids 1975;25(3):365-78.
1974: Stephen G Tint; G Salen
Transformation of 5 alpha-cholest-7-en-3 beta-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis.
Journal of lipid research 1974;15(3):256-62.
1974: T Setoguchi; G Salen; Stephen G Tint; E H Mosbach
A biochemical abnormality in cerebrotendinous xanthomatosis. Impairment of bile acid biosynthesis associated with incomplete degradation of the cholesterol side chain.
The Journal of clinical investigation 1974;53(5):1393-401.
1974: G Salen; Stephen G Tint; B Eliav; N Deering; E H Mosbach
Increased formation of ursodeoxycholic acid in patients treated with chenodeoxycholic acid.
The Journal of clinical investigation 1974;53(2):612-21.