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Sarah Shefer

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Ashok K Batta; Guorong Xu; Jaya Bollineni; Sarah Shefer; Gerald Salen
Effect of high plant sterol-enriched diet and cholesterol absorption inhibitor, SCH 58235, on plant sterol absorption and plasma concentrations in hypercholesterolemic wild-type Kyoto rats.
Sandrine Dubrac; Steven R Lear; Meenakshisundaram Ananthanarayanan; Natarajan Balasubramaniyan; Jaya Bollineni; Sarah Shefer; Hideyuki Hyogo; David E Cohen; Patricia J Blanche; Ronald M Krauss; Ashok K Batta; Gerald Salen; Frederick J Suchy; Nobuyo Maeda; Sandra K Erickson
Role of CYP27A in cholesterol and bile acid metabolism.
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.

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2005: Ashok K Batta; Guorong Xu; Jaya Bollineni; Sarah Shefer; Gerald Salen
Effect of high plant sterol-enriched diet and cholesterol absorption inhibitor, SCH 58235, on plant sterol absorption and plasma concentrations in hypercholesterolemic wild-type Kyoto rats.
Metabolism: clinical and experimental 2005;54(1):38-48.
2005: Sandrine Dubrac; Steven R Lear; Meenakshisundaram Ananthanarayanan; Natarajan Balasubramaniyan; Jaya Bollineni; Sarah Shefer; Hideyuki Hyogo; David E Cohen; Patricia J Blanche; Ronald M Krauss; Ashok K Batta; Gerald Salen; Frederick J Suchy; Nobuyo Maeda; Sandra K Erickson
Role of CYP27A in cholesterol and bile acid metabolism.
Journal of lipid research 2005;46(1):76-85.
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: 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: Michaela Tiemann; Zhihua Han; Raymond E Soccio; Jaya Bollineni; Sarah Shefer; Ephraim Sehayek; Jan L Breslow
Cholesterol feeding of mice expressing cholesterol 7alpha-hydroxylase increases bile acid pool size despite decreased enzyme activity.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(7):1846-51.
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: Ephraim Sehayek; Rong Wang; Jennie G Ono; Vadim S Zinchuk; Elizabeth M Duncan; Sarah Shefer; Dennis E Vance; Meenakshisundaram Ananthanarayanan; Brian T Chait; Jan L Breslow
Localization of the PE methylation pathway and SR-BI to the canalicular membrane: evidence for apical PC biosynthesis that may promote biliary excretion of phospholipid and cholesterol.
Journal of lipid research 2003;44(9):1605-13.
2003: Sandra K Erickson; Steven R Lear; Sean Deane; Sandrine Dubrac; Sandra L Huling; Lien Nguyen; Jaya Bollineni; Sarah Shefer; Hideyuki Hyogo; David E Cohen; Benjamin L Shneider; Ephraim Sehayek; Meenakshisundaram Ananthanarayanan; Natarajan Balasubramaniyan; Frederick J Suchy; Ashok K Batta; Gerald Salen
Hypercholesterolemia and changes in lipid and bile acid metabolism in male and female cyp7A1-deficient mice.
Journal of lipid research 2003;44(5):1001-9.
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: Namir Al-Ansari; Guorong Xu; Katie Kollman-Bauerly; Christopher P Coppola; Sarah Shefer; P Ujházy; Daniel Ortiz; Lin Ma; Steven Yang; Richard Tsai; Gerald Salen; Jon A Vanderhoof; Benjamin L Shneider
Analysis of the effect of intestinal resection on rat ileal bile Acid transporter expression and on bile Acid and cholesterol homeostasis.
Pediatric research 2002;52(2):286-91.
2002: Clive R Pullinger; Celeste Eng; Gerald Salen; Sarah Shefer; Ashok K Batta; Sandra K Erickson; Andrea Verhagen; Christopher R Rivera; Sean J Mulvihill; Mary J Malloy; John P Kane
Human cholesterol 7alpha-hydroxylase (CYP7A1) deficiency has a hypercholesterolemic phenotype.
The Journal of clinical investigation 2002;110(1):109-17.
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: Mohammed H Moghadasian; Bruce M McManus; Lien B Nguyen; Sarah Shefer; Mehrdad Nadji; David V Godin; T J Green; J Hill; Y Yang; Charles H Scudamore; Jiri J Frohlich
Pathophysiology of apolipoprotein E deficiency in mice: relevance to apo E-related disorders in humans.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2001;15(14):2623-30.
2001: Thomas P Johnston; Lien B Nguyen; W A Chu; Sarah Shefer
Potency of select statin drugs in a new mouse model of hyperlipidemia and atherosclerosis.
International journal of pharmaceutics 2001;229(1-2):75-86.
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: Ephraim Sehayek; Jennie G Ono; Elizabeth M Duncan; Ashok K Batta; Gerald Salen; Sarah Shefer; L B Neguyen; Kan Yang; Martin Lipkin; Jan L Breslow
Hyodeoxycholic acid efficiently suppresses atherosclerosis formation and plasma cholesterol levels in mice.
Journal of lipid research 2001;42(8):1250-6.
2001: Sonya Vanpatten; N Ranginani; Sarah Shefer; Lien B Nguyen; Luciano Rossetti; David E Cohen
Impaired biliary lipid secretion in obese Zucker rats: leptin promotes hepatic cholesterol clearance.
American journal of physiology. Gastrointestinal and liver physiology 2001;281(2):G393-404.
2001: Mohammed H Moghadasian; Lien B Nguyen; Sarah Shefer; Gerald Salen; Ashok K Batta; Jiri J Frohlich
Hepatic cholesterol and bile acid synthesis, low-density lipoprotein receptor function, and plasma and fecal sterol levels in mice: effects of apolipoprotein E deficiency and probucol or phytosterol treatment.
Metabolism: clinical and experimental 2001;50(6):708-14.
2001: D Q Shih; M Bussen; Ephraim Sehayek; Meenakshisundaram Ananthanarayanan; Benjamin L Shneider; Frederick J Suchy; Sarah Shefer; J S Bollileni; F J Gonzalez; Jan L Breslow; Markus Stoffel
Hepatocyte nuclear factor-1alpha is an essential regulator of bile acid and plasma cholesterol metabolism.
Nature genetics 2001;27(4):375-82.
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: 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: 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: Ephraim Sehayek; Sarah Shefer; Lien B Nguyen; Jennie G Ono; Martin Merkel; Jan L Breslow
Apolipoprotein E regulates dietary cholesterol absorption and biliary cholesterol excretion: studies in C57BL/6 apolipoprotein E knockout mice.
Proceedings of the National Academy of Sciences of the United States of America 2000;97(7):3433-7.
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: 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: 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: Mohammed H Moghadasian; Lien B Nguyen; Sarah Shefer; Bruce M McManus; Jiri J Frohlich
Histologic, hematologic, and biochemical characteristics of apo E-deficient mice: effects of dietary cholesterol and phytosterols.
Laboratory investigation; a journal of technical methods and pathology 1999;79(3):355-64.
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: 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: Ephraim Sehayek; Jennie G Ono; Sarah Shefer; Lien B Nguyen; N Wang; Ashok K Batta; Gerald Salen; Jonathan D. Smith; Alan R Tall; Jan L Breslow
Biliary cholesterol excretion: a novel mechanism that regulates dietary cholesterol absorption.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(17):10194-9.
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: 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: 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: 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: 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: 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: Gerald Salen; Sarah Shefer; L Nguyen; G C Ness; Stephen G Tint; Ashok K Batta
Sitosterolemia.
Sub-cellular biochemistry 1997;28():453-76.
1996: Lien B Nguyen; Sarah Shefer; G Salen; J Y Chiang; M Patel
Cholesterol 7alpha-hydroxylase activities from human and rat liver are modulated in vitro posttranslationally by phosphorylation/dephosphorylation.
Hepatology (Baltimore, Md.) 1996;24(6):1468-74.
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: 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: Bishambar Dayal; J Bhojawala; KR Rapole; B N Pramanik; Norman H Ertel; Sarah Shefer; G Salen
Chemical synthesis, structural analysis, and decomposition of N-nitroso bile acid conjugates.
Bioorganic & medicinal chemistry 1996;4(6):885-90.
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.
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: 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: 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.
1994: Lien B Nguyen; Sarah Shefer; G Salen; G C Ness; A Batta; I R Chowdhary; E Paroulek; S Hauser
Regulation of 3-hydroxy-3-methylglutaryl-coenzyme A reductase activity in the rat ileum: effects of bile acids and lovastatin.
Metabolism: clinical and experimental 1994;43(11):1446-50.
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: Sarah Shefer; G Salen; J Bullock; Lien B Nguyen; G C Ness; Z Vhao; Peter F Belamarich; I Chowdhary; S Lerner; A K Batta
The effect of increased hepatic sitosterol on the regulation of 3-hydroxy-3-methylglutaryl-coenzyme A reductase and cholesterol 7 alpha-hydroxylase in the rat and sitosterolemic homozygotes.
Hepatology (Baltimore, Md.) 1994;20(1 Pt 1):213-9.
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: 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.
1992: A K Batta; S K Aggarwal; R Mirchandani; Sarah Shefer; G Salen
Capillary gas-liquid chromatographic separation of bile alcohols.
Journal of lipid research 1992;33(9):1403-7.
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: 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: A K Batta; R Mirchandani; G Salen; Sarah Shefer
Synthesis of 3 alpha, 7 alpha-dihydroxy-5 beta-cholestan-26-oic acid from 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestan-26-oic acid: configuration in the bile of Alligator mississippiensis.
Steroids 1992;57(4):162-6.
1992: S K Aggarwal; A K Batta; G Salen; Sarah Shefer
Synthesis of 3 alpha,6 beta,7 alpha,12 beta- and 3 alpha,6 beta,7 beta,12 beta-tetrahydroxy-5 beta-cholanoic acids.
Steroids 1992;57(3):107-11.
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: A K Batta; S K Aggarwal; G Salen; Sarah Shefer
Selective reduction of oxo bile acids: synthesis of 3 beta-, 7 beta-, and 12 beta-hydroxy bile acids.
Journal of lipid research 1991;32(6):977-83.
1991: A K Batta; G Salen; R Arora; Sarah Shefer; M Batta
High-performance liquid chromatographic separation of bile acids and bile alcohols diastereoisomeric at C-25.
Journal of chromatography 1991;542(1):184-8.
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: 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: A K Batta; G Salen; R Arora; Sarah Shefer; M Batta; A Person
Side chain conjugation prevents bacterial 7-dehydroxylation of bile acids.
The Journal of biological chemistry 1990;265(19):10925-8.
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: Lien B Nguyen; Sarah Shefer; G Salen; G Ness; R D Tanaka; V Packin; P Thomas; V Shore; A Batta
Purification of cholesterol 7 alpha-hydroxylase from human and rat liver and production of inhibiting polyclonal antibodies.
The Journal of biological chemistry 1990;265(8):4541-6.
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; 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.
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: A K Batta; G Salen; Sarah Shefer
Chromatographic separation of putative precursors of cholestanol.
Steroids 1988;52(1-2):109-22.
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: 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: 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: A K Batta; Sarah Shefer; M Batta; G Salen
Effect of chenodeoxycholic acid on biliary and urinary bile acids and bile alcohols in cerebrotendinous xanthomatosis; monitoring by high performance liquid chromatography.
Journal of lipid research 1985;26(6):690-8.
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.
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.
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: 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.