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Paul Watkins

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Christa Montgomery; Zhengtong Pei; Paul A Watkins; Henry M Miziorko
Identification and characterization of an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase.
Paul A Watkins; Jessica M Ellis
Peroxisomal acyl-CoA synthetases.
Shilpa D Kadam; Paul A Watkins; Robert N Cole; Marjan Gucek; Anne M Comi
Cell proliferation and oxidative stress pathways are modified in fibroblasts from Sturge-Weber syndrome patients.

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All Publications

2012: Christa Montgomery; Zhengtong Pei; Paul A Watkins; Henry M Miziorko
Identification and characterization of an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase.
The Journal of biological chemistry 2012;287(40):33227-36.
2012: Paul A Watkins; Jessica M Ellis
Peroxisomal acyl-CoA synthetases.
Biochimica et biophysica acta 2012;1822(9):1411-20.
2012: Shilpa D Kadam; Paul A Watkins; Robert N Cole; Marjan Gucek; Anne M Comi
Cell proliferation and oxidative stress pathways are modified in fibroblasts from Sturge-Weber syndrome patients.
Archives of dermatological research 2012;304(3):229-35.
2011: Elaina M Melton; Paul A Watkins; Ronald L Cerny; Concetta C DiRusso; Paul N Black
Human fatty acid transport protein 2a/very long chain acyl-CoA synthetase 1 (FATP2a/Acsvl1) has a preference in mediating the channeling of exogenous n-3 fatty acids into phosphatidylinositol.
The Journal of biological chemistry 2011;286(35):30670-9.
2011: Ying Wang; Paul A Watkins; Gail Stetten; Karen Tam; Cicely J Toomer; Lena Bezman; Genila Bibat; Rachel Busin; Lawrence Charnas; Joe T R Clarke; Garry R Cutting; Stacy Hewson; Barbara Karczeski; Ann B Moser; Sakkubai Naidu; Gerald V Raymond; Catherine Reeves; Ann Snowden; Steven J Steinberg
X-linked adrenoleukodystrophy: ABCD1 de novo mutations and mosaicism.
Molecular genetics and metabolism 2011;104(1-2):160-6.
2011: Miao He; Zhengtong Pei; Al-Walid Mohsen; Paul A Watkins; Geoffrey Murdoch; Paul P Van Veldhoven; Regina Ensenauer; Jerry Vockley
Identification and characterization of new long chain acyl-CoA dehydrogenases.
Molecular genetics and metabolism 2011;102(4):418-29.
2011: Ann B Moser; Hugo W Moser; Krishna Ramaswamy; Oliver A Ryder; Kimberly D Siegmund; Steven J Steinberg; Paul A Watkins; John J Ely; D Rick Lee; Joseph G Hacia
Human and great ape red blood cells differ in plasmalogen levels and composition.
Lipids in health and disease 2011;10():101.
2010: Paul A Watkins; Ann B Moser; Cicely B Toomer; Steven J Steinberg; Hugo W Moser; Mazen W Karaman; Krishna Ramaswamy; Kimberly D Siegmund; D Rick Lee; John J Ely; Oliver A Ryder; Joseph G Hacia
Identification of differences in human and great ape phytanic acid metabolism that could influence gene expression profiles and physiological functions.
BMC physiology 2010;10():19.
2009: Zhengtong Pei; Peng Sun; Peng Huang; Bachchu Lal; John Laterra; Paul A Watkins
Acyl-CoA synthetase VL3 knockdown inhibits human glioma cell proliferation and tumorigenicity.
Cancer research 2009;69(24):9175-82.
2009: Steven J Steinberg; A Snowden; Nancy Braverman; L Chen; Paul A Watkins; P T Clayton; K D R Setchell; James Heubi; Gerald V Raymond; Ann B Moser; Hugo W Moser
A PEX10 defect in a patient with no detectable defect in peroxisome assembly or metabolism in cultured fibroblasts.
Journal of inherited metabolic disease 2009;32(1):109-19.
2009: Lisa M Davis; Michael Michaelides; Lawrence J Cheskin; Timothy H Moran; Susan Aja; Paul A Watkins; Zhengtong Pei; Carlo Contoreggi; Karen McCullough; Bruce Hope; Gene-Jack Wang; Nora D Volkow; Panayotis K Thanos
Bromocriptine administration reduces hyperphagia and adiposity and differentially affects dopamine D2 receptor and transporter binding in leptin-receptor-deficient Zucker rats and rats with diet-induced obesity.
Neuroendocrinology 2009;89(2):152-62.
2008: Paul A Watkins
Very-long-chain acyl-CoA synthetases.
The Journal of biological chemistry 2008;283(4):1773-7.
2007: Paul A Watkins; Dony Maiguel; Zhenzhen Jia; Jonathan Pevsner
Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome.
Journal of lipid research 2007;48(12):2736-50.
2007: Robert Katz; James A Hamilton; Henry J Pownall; Richard J Deckelbaum; Cecilia J Hillard; Renee C Leboeuf; Paul A Watkins
Brain uptake and utilization of fatty acids, lipids & lipoproteins: recommendations for future research.
Journal of molecular neuroscience : MN 2007;33(1):146-50.
2007: Paul A Watkins; James A Hamilton
Roundtable discussion of session 4: fatty acids and lipids in brain disorders.
Journal of molecular neuroscience : MN 2007;33(1):120-4.
2007: Paul A Watkins; James A Hamilton
Roundtable discussion of session 3: eicosanoids in brain function.
Journal of molecular neuroscience : MN 2007;33(1):100-4.
2007: Paul A Watkins; James A Hamilton
Roundtable discussion of session 2: lipoproteins and polyunsaturated fatty acids.
Journal of molecular neuroscience : MN 2007;33(1):74-9.
2007: Paul A Watkins; James A Hamilton
Roundtable discussion of session 1: mechanisms of lipid uptake and transport in the brain.
Journal of molecular neuroscience : MN 2007;33(1):45-50.
2007: Zhenzhen Jia; Zhengtong Pei; Dony Maiguel; Cicely J Toomer; Paul A Watkins
The fatty acid transport protein (FATP) family: very long chain acyl-CoA synthetases or solute carriers?
Journal of molecular neuroscience : MN 2007;33(1):25-31.
2007: James A Hamilton; Cecilia J Hillard; Arthur A Spector; Paul A Watkins
Brain uptake and utilization of fatty acids, lipids and lipoproteins: application to neurological disorders.
Journal of molecular neuroscience : MN 2007;33(1):2-11.
2007: Zhenzhen Jia; Casey L Moulson; Zhengtong Pei; Jeffrey H Miner; Paul A Watkins
Fatty acid transport protein 4 is the principal very long chain fatty acyl-CoA synthetase in skin fibroblasts.
The Journal of biological chemistry 2007;282(28):20573-83.
2007: M He; S L Rutledge; David R Kelly; Cheryl Ann Palmer; G Murdoch; N Majumder; R D Nicholls; Zhengtong Pei; Paul A Watkins; Jerry Vockley
A new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiency.
American journal of human genetics 2007;81(1):87-103.
2007: Jyh-Feng Lu; EMILY BARRON-CASELLA; Rebecca Deering; Ann Heinzer; Ann B Moser; Karen L deMesy Bentley; Gary S Wand; Martina C McGuinness; Zhengtong Pei; Paul A Watkins; Aurora Pujol; Kirby D Smith; James M Powers
The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage.
Laboratory investigation; a journal of technical methods and pathology 2007;87(3):261-72.
2006: Katrin Milger; Thomas Herrmann; Christiane Becker; Daniel Gotthardt; Jelena Zickwolf; Robert Ehehalt; Paul A Watkins; Wolfgang Stremmel; Joachim Füllekrug
Cellular uptake of fatty acids driven by the ER-localized acyl-CoA synthetase FATP4.
Journal of cell science 2006;119(Pt 22):4678-88.
2006: Lisa M Davis; Zhengtong Pei; Michael A Trush; Lawrence J Cheskin; Carlo Contoreggi; Karen McCullough; Paul A Watkins; Timothy H Moran
Bromocriptine reduces steatosis in obese rodent models.
Journal of hepatology 2006;45(3):439-44.
2006: Zhengtong Pei; Zhenzhen Jia; Paul A Watkins
The second member of the human and murine bubblegum family is a testis- and brainstem-specific acyl-CoA synthetase.
The Journal of biological chemistry 2006;281(10):6632-41.
2005: James M Powers; Zhengtong Pei; Ann Heinzer; Rebecca Deering; Ann B Moser; Hugo W Moser; Paul A Watkins; Kirby D Smith
Adreno-leukodystrophy: oxidative stress of mice and men.
Journal of neuropathology and experimental neurology 2005;64(12):1067-79.
2005: Concetta C DiRusso; Hong Li; Dina Darwis; Paul A Watkins; Johannas Berger; Paul N Black
Comparative biochemical studies of the murine fatty acid transport proteins (FATP) expressed in yeast.
The Journal of biological chemistry 2005;280(17):16829-37.
2004: Zhengtong Pei; Peter Fraisl; Johannes Berger; Zhenzhen Jia; Sonja Forss-Petter; Paul A Watkins
Mouse very long-chain Acyl-CoA synthetase 3/fatty acid transport protein 3 catalyzes fatty acid activation but not fatty acid transport in MA-10 cells.
The Journal of biological chemistry 2004;279(52):54454-62.
2004: Douglas G Mashek; Karin E Bornfeldt; Rosalind A Coleman; Johannes Berger; David A Bernlohr; Paul Black; Concetta C DiRusso; Steven A Farber; Wen Guo; Naohiro Hashimoto; Varsha K Khodiyar; Frans A Kuypers; Lois J Maltais; Daniel W Nebert; Alessandra Renieri; Jean E Schaffer; Andreas Stahl; Paul A Watkins; Vasilis Vasiliou; Tokuo T Yamamoto
Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.
Journal of lipid research 2004;45(10):1958-61.
2004: Zhenzhen Jia; Zhengtong Pei; Yuanyuan Li; Liumei Wei; Kirby D Smith; Paul A Watkins
X-linked adrenoleukodystrophy: role of very long-chain acyl-CoA synthetases.
Molecular genetics and metabolism 2004;83(1-2):117-27.
2003: Zhengtong Pei; Nadia A Oey; Maartje M Zuidervaart; Zhenzhen Jia; Yuanyuan Li; Steven J Steinberg; Kirby D Smith; Paul A Watkins
The acyl-CoA synthetase "bubblegum" (lipidosin): further characterization and role in neuronal fatty acid beta-oxidation..
The Journal of biological chemistry 2003;278(47):47070-8.
2003: Ann Heinzer; Paul A Watkins; Jyh-Feng Lu; Stephan Kemp; Ann B Moser; Yuan Yuan Li; Stephanie J Mihalik; James M Powers; Kirby D Smith
A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy.
Human molecular genetics 2003;12(10):1145-54.
2003: Zhiping Li; Shiqi Yang; Huizhi Lin; Jiawen Huang; Paul A Watkins; Ann B Moser; Claudio Desimone; Xiao-yu Song; Anna Mae Diehl
Probiotics and antibodies to TNF inhibit inflammatory activity and improve nonalcoholic fatty liver disease.
Hepatology (Baltimore, Md.) 2003;37(2):343-50.
2003: Ann Heinzer; Martina C McGuinness; Jyh-Feng Lu; O Colin Stine; Heming Wei; Mark Van der Vlies; Gao-xiang Dong; James Powers; Paul A Watkins; Kirby D Smith
Mouse models and genetic modifiers in X-linked adrenoleukodystrophy.
Advances in experimental medicine and biology 2003;544():75-93.
2003: Martina C McGuinness; Jyh-Feng Lu; H-P Zhang; Gao-xiang Dong; Ann Heinzer; Paul A Watkins; J Powers; Kirby D Smith
Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy.
Molecular and cellular biology 2003;23(2):744-53.
2002: Ann Heinzer; Stephan Kemp; Jyh-Feng Lu; Paul A Watkins; Kirby D Smith
Mouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophy.
The Journal of biological chemistry 2002;277(32):28765-73.
2002: Eli Hershkovitz; Ginat Narkis; Zamir Shorer; Ann B Moser; Paul A Watkins; Hugo W Moser; Esther Manor
Cerebral X-linked adrenoleukodystrophy in a girl with Xq27-Ter deletion.
Annals of neurology 2002;52(2):234-7.
2002: Stephanie J Mihalik; Steven J Steinberg; Zhengtong Pei; Joseph Park; Do G Kim; Ann Heinzer; Georges Dacremont; Ronald J A Wanders; Dean Cuebas; Kirby D Smith; Paul A Watkins
Participation of two members of the very long-chain acyl-CoA synthetase family in bile acid synthesis and recycling.
The Journal of biological chemistry 2002;277(27):24771-9.
2002: Deyanira Corzo; William Gibson; Kisha Johnson; Grant Mitchell; Guy LePage; Gerald F Cox; Robin Casey; Carolyn Zeiss; Heidi Tyson; Garry R Cutting; Gerald V Raymond; Kirby D Smith; Paul A Watkins; Ann B Moser; Hugo W Moser; Steven J Steinberg
Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: a novel neonatal phenotype similar to peroxisomal biogenesis disorders.
American journal of human genetics 2002;70(6):1520-31.
2001: H M Su; Ann B Moser; Hugo W Moser; Paul A Watkins
Peroxisomal straight-chain Acyl-CoA oxidase and D-bifunctional protein are essential for the retroconversion step in docosahexaenoic acid synthesis.
The Journal of biological chemistry 2001;276(41):38115-20.
2001: Paul A Watkins; James A Hamilton; A Leaf; A A Spector; S A Moore; R E Anderson; Hugo W Moser; M J Noetzel; R Katz
Brain uptake and utilization of fatty acids: applications to peroxisomal biogenesis diseases.
Journal of molecular neuroscience : MN 2001;16(2-3):87-92; discussion 151-7.
2001: R Katz; J A Hamilton; A A Spector; S A Moore; H W Moser; M J Noetzel; Paul A Watkins
Brain uptake and utilization of fatty acids: recommendations for future research.
Journal of molecular neuroscience : MN 2001;16(2-3):333-5.
2001: Lena Bezman; Ann B Moser; Gerald V Raymond; P Rinaldo; Paul A Watkins; Kirby D Smith; Nancy Kass; Hugo W Moser
Adrenoleukodystrophy: incidence, new mutation rate, and results of extended family screening.
Annals of neurology 2001;49(4):512-7.
2000: Steven J Steinberg; J Morgenthaler; Ann Heinzer; Kirby D Smith; Paul A Watkins
Very long-chain acyl-CoA synthetases. Human "bubblegum" represents a new family of proteins capable of activating very long-chain fatty acids.
The Journal of biological chemistry 2000;275(45):35162-9.
2000: Steven J Steinberg; Stephanie J Mihalik; Do G Kim; Dean Cuebas; Paul A Watkins
The human liver-specific homolog of very long-chain acyl-CoA synthetase is cholate:CoA ligase.
The Journal of biological chemistry 2000;275(21):15605-8.
2000: Paul A Watkins; Jyh-Feng Lu; Lelita T Braiterman; Steven J Steinberg; Kirby D Smith
Disruption of a yeast very-long-chain acyl-CoA synthetase gene simulates the cellular phenotype of X-linked adrenoleukodystrophy.
Cell biochemistry and biophysics 2000;32 Spring():333-7.
1999: NR Coe; Anne J Smith; BI Frohnert; Paul A Watkins; David A Bernlohr
The fatty acid transport protein (FATP1) is a very long chain acyl-CoA synthetase.
The Journal of biological chemistry 1999;274(51):36300-4.
1999: Omer N Koç; Charles Peters; Patrick R Aubourg; S Raghavan; S Dyhouse; Rita DeGasperi; E H Kolodny; Y B Yoseph; Stanton L Gerson; H M Lazarus; Arnold I Caplan; Paul A Watkins; William Krivit
Bone marrow-derived mesenchymal stem cells remain host-derived despite successful hematopoietic engraftment after allogeneic transplantation in patients with lysosomal and peroxisomal storage diseases.
Experimental hematology 1999;27(11):1675-81.
1999: Steven J Steinberg; Stephan Kemp; Lelita T Braiterman; Paul A Watkins
Role of very-long-chain acyl-coenzyme A synthetase in X-linked adrenoleukodystrophy.
Annals of neurology 1999;46(3):409-12.
1999: Steven J Steinberg; S J Wang; Martina C McGuinness; Paul A Watkins
Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.
Molecular genetics and metabolism 1999;68(1):32-42.
1999: Paul A Watkins; Jonathan Pevsner; Steven J Steinberg
Human very long-chain acyl-CoA synthetase and two human homologs: initial characterization and relationship to fatty acid transport protein.
Prostaglandins, leukotrienes, and essential fatty acids 1999;60(5-6):323-8.
1999: Steven J Steinberg; S J Wang; Do G Kim; Stephanie J Mihalik; Paul A Watkins
Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism.
Biochemical and biophysical research communications 1999;257(2):615-21.
1999: Kirby D Smith; Stephan Kemp; Lelita T Braiterman; Jyh-Feng Lu; H M Wei; M Geraghty; Gail Stetten; J S Bergin; Jonathan Pevsner; Paul A Watkins
X-linked adrenoleukodystrophy: genes, mutations, and phenotypes.
Neurochemical research 1999;24(4):521-35.
1999: Lelita T Braiterman; Paul A Watkins; Ann B Moser; Kirby D Smith
Peroxisomal very long chain fatty acid beta-oxidation activity is determined by the level of adrenodeukodystrophy protein (ALDP) expression.
Molecular genetics and metabolism 1999;66(2):91-9.
1999: Elisabeth G van Grunsven; E van Berkel; Petra A W Mooijer; Paul A Watkins; Hugo W Moser; Yasuyuki Suzuki; L L Jiang; T Hashimoto; Gerald Hoefler; Jerzy Adamski; Ronald J A Wanders
Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
American journal of human genetics 1999;64(1):99-107.
1998: Stephan Kemp; H M Wei; Jyh-Feng Lu; Lelita T Braiterman; Martina C McGuinness; Ann B Moser; Paul A Watkins; Kirby D Smith
Gene redundancy and pharmacological gene therapy: implications for X-linked adrenoleukodystrophy.
Nature medicine 1998;4(11):1261-8.
1998: Jutta Gärtner; Ute Brosius; C Obie; Paul A Watkins; David Valle
Restoration of PEX2 peroxisome assembly defects by overexpression of PMP70.
European journal of cell biology 1998;76(4):237-45.
1998: Paul A Watkins; Jyh-Feng Lu; Steven J Steinberg; Stephen J Gould; Kirby D Smith; Lelita T Braiterman
Disruption of the Saccharomyces cerevisiae FAT1 gene decreases very long-chain fatty acyl-CoA synthetase activity and elevates intracellular very long-chain fatty acid concentrations.
The Journal of biological chemistry 1998;273(29):18210-9.
1998: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Cornelis Jakobs; Hugo W Moser; Ronald J A Wanders
Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.
Clinica chimica acta; international journal of clinical chemistry 1998;271(2):203-11.
1998: S M L C Mendis-Handagama; Paul A Watkins; S J Gelber; T J Scallen
The effect of chronic luteinizing hormone treatment on adult rat Leydig cells.
Tissue & cell 1998;30(1):64-73.
1998: Lelita T Braiterman; S Zheng; Paul A Watkins; M T Geraghty; G Johnson; Martina C McGuinness; Ann B Moser; Kirby D Smith
Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins.
Human molecular genetics 1998;7(2):239-47.
1997: Stephanie J Mihalik; James C Morrell; D Kim; Katherine A Sacksteder; Paul A Watkins; Stephen J Gould
Identification of PAHX, a Refsum disease gene.
Nature genetics 1997;17(2):185-9.
1997: Jyh-Feng Lu; Ann M Lawler; Paul A Watkins; James M Powers; Ann B Moser; Hugo W Moser; Kirby D Smith
A mouse model for X-linked adrenoleukodystrophy.
Proceedings of the National Academy of Sciences of the United States of America 1997;94(17):9366-71.
1997: Gerbert Jansen; Ronald J A Wanders; Paul A Watkins; Stephanie J Mihalik
Phytanoyl-coenzyme A hydroxylase deficiency -- the enzyme defect in Refsum's disease.
The New England journal of medicine 1997;337(2):133-4.
1997: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Hugo W Moser; Cornelis Jakobs; Hugo S A Heijmans; Ronald J A Wanders
Phytanoyl-CoA hydroxylase is not only deficient in classical Refsum disease but also in rhizomelic chondrodysplasia punctata.
Journal of inherited metabolic disease 1997;20(3):444-6.
1997: Paul A Watkins
Fatty acid activation.
Progress in lipid research 1997;36(1):55-83.
1996: N Shani; A Sapag; Paul A Watkins; David Valle
An S. cerevisiae peroxisomal transporter, orthologous to the human adrenoleukodystrophy protein, appears to be a heterodimer of two half ABC transporters: Pxa1p and Pxa2p.
Annals of the New York Academy of Sciences 1996;804():770-2.
1996: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Hugo W Moser; C Jakobs; Simone Denis; R J Wanders
Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.
Biochemical and biophysical research communications 1996;229(1):205-10.
1996: Paul A Watkins; A E Howard; Stephen J Gould; J Avigan; Stephanie J Mihalik
Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase.
Journal of lipid research 1996;37(11):2288-95.
1996: M R Natowicz; J E Evans; Richard I Kelley; Ann B Moser; Paul A Watkins; H W Moser
Urinary bile acids and peroxisomal bifunctional enzyme deficiency.
American journal of medical genetics 1996;63(2):356-62.
1996: Walter E Kaufmann; CHRISTIANE THEDA; Sakkubai Naidu; Paul A Watkins; Ann B Moser; Hugo W Moser
Neuronal migration abnormality in peroxisomal bifunctional enzyme defect.
Annals of neurology 1996;39(2):268-71.
1995: S M L C Mendis-Handagama; R F Aten; Paul A Watkins; T J Scallen; H R Berhman
Peroxisomes and sterol carrier protein-2 in luteal cell steroidogenesis: a possible role in cholesterol transport from lipid droplets to mitochondria.
Tissue & cell 1995;27(5):483-90.
1995: Paul A Watkins; Martina C McGuinness; Gerald V Raymond; B A Hicks; Jeanne Sisk; Ann B Moser; Hugo W Moser
Distinction between peroxisomal bifunctional enzyme and acyl-CoA oxidase deficiencies.
Annals of neurology 1995;38(3):472-7.
1995: Stephanie J Mihalik; A M Rainville; Paul A Watkins
Phytanic acid alpha-oxidation in rat liver peroxisomes. Production of alpha-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors.
European journal of biochemistry / FEBS 1995;232(2):545-51.
1995: Paul A Watkins; Stephen J Gould; M A Smith; Lelita T Braiterman; H M Wei; F Kok; Ann B Moser; Hugo W Moser; Kirby D Smith
Altered expression of ALDP in X-linked adrenoleukodystrophy.
American journal of human genetics 1995;57(2):292-301.
1995: Ann B Moser; M Rasmussen; Sakkubai Naidu; Paul A Watkins; Martina C McGuinness; A K Hajra; G Chen; Gerald V Raymond; A Liu; D Gordon
Phenotype of patients with peroxisomal disorders subdivided into sixteen complementation groups.
The Journal of pediatrics 1995;127(1):13-22.
1995: N Shani; Paul A Watkins; David Valle
PXA1, a possible Saccharomyces cerevisiae ortholog of the human adrenoleukodystrophy gene.
Proceedings of the National Academy of Sciences of the United States of America 1995;92(13):6012-6.
1995: S Hosain; Walter E Kaufmann; G Negrin; Paul A Watkins; A N Siakotos; D N Palmer; Sakkubai Naidu
Diagnoses of neuronal ceroid-lipofuscinosis by immunochemical methods.
American journal of medical genetics 1995;57(2):239-45.
1995: Gabriele Dodt; Nancy Braverman; C Wong; Ann B Moser; Hugo W Moser; Paul A Watkins; David Valle; Stephen J Gould
Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders.
Nature genetics 1995;9(2):115-25.
1995: J A Gordon; S K Heller; W J Rhead; Paul A Watkins; Arthur A Spector
Formation of a novel arachidonic acid metabolite in peroxisomes.
Prostaglandins, leukotrienes, and essential fatty acids 1995;52(2-3):77-81.
1995: J E Kalish; C I Chen; Stephen J Gould; Paul A Watkins
Peroxisomal activation of long- and very long-chain fatty acids in the yeast Pichia pastoris.
Biochemical and biophysical research communications 1995;206(1):335-40.
1995: F Kok; S Neumann; C O Sarde; S Zheng; K H Wu; H M Wei; J Bergin; Paul A Watkins; Stephen J Gould; GEORGE SACK
Mutational analysis of patients with X-linked adrenoleukodystrophy.
Human mutation 1995;6(2):104-15.
1995: William Krivit; L A Lockman; Paul A Watkins; J Hirsch; Elsa G Shapiro
The future for treatment by bone marrow transplantation for adrenoleukodystrophy, metachromatic leukodystrophy, globoid cell leukodystrophy and Hurler syndrome.
Journal of inherited metabolic disease 1995;18(4):398-412.
1994: Paul A Watkins; A E Howard; Stephanie J Mihalik
Phytanic acid must be activated to phytanoyl-CoA prior to its alpha-oxidation in rat liver peroxisomes.
Biochimica et biophysica acta 1994;1214(3):288-94.
1994: Jutta Gärtner; Cassandra Obie; Paul A Watkins; David Valle
Restoration of peroxisome biogenesis in a peroxisome-deficient mammalian cell line by expression of either the 35 kDa or the 70 kDa peroxisomal membrane proteins.
Journal of inherited metabolic disease 1994;17(3):327-9.
1993: Martina C McGuinness; Ann B Moser; B T Poll-The; Paul A Watkins
Complementation analysis of patients with intact peroxisomes and impaired peroxisomal beta-oxidation.
Biochemical medicine and metabolic biology 1993;49(2):228-42.
1992: S M L C Mendis-Handagama; Paul A Watkins; S J Gelber; T J Scallen
Leydig cell peroxisomes and sterol carrier protein-2 in luteinizing hormone-deprived rats.
Endocrinology 1992;131(6):2839-45.
1992: Stephanie J Mihalik; T M Soliman; R F Day; Paul A Watkins
Involvement of both peroxisomes and mitochondria in the alpha-oxidation of phytanic acid.
Progress in clinical and biological research 1992;375():239-44.
1991: Paul A Watkins; E V Ferrell; J I Pedersen; Gerald Hoefler
Peroxisomal fatty acid beta-oxidation in HepG2 cells.
Archives of biochemistry and biophysics 1991;289(2):329-36.
1991: Stephanie J Mihalik; Martina C McGuinness; Paul A Watkins
Purification and characterization of peroxisomal L-pipecolic acid oxidase from monkey liver.
The Journal of biological chemistry 1991;266(8):4822-30.
1990: S M L C Mendis-Handagama; Paul A Watkins; S J Gelber; T J Scallen; Barry R Zirkin; L L Ewing
Luteinizing hormone causes rapid and transient changes in rat Leydig cell peroxisome volume and intraperoxisomal sterol carrier protein-2 content.
Endocrinology 1990;127(6):2947-54.
1990: Martina C McGuinness; Ann B Moser; Hugo W Moser; Paul A Watkins
Peroxisomal disorders: complementation analysis using beta-oxidation of very long chain fatty acids.
Biochemical and biophysical research communications 1990;172(1):364-9.
1990: Paul A Watkins; Stephanie J Mihalik
Mitochondrial oxidation of phytanic acid in human and monkey liver: implication that Refsum's disease is not a peroxisomal disorder.
Biochemical and biophysical research communications 1990;167(2):580-6.
1990: A Balfe; Gerald Hoefler; W W Chen; Paul A Watkins
Aberrant subcellular localization of peroxisomal 3-ketoacyl-CoA thiolase in the Zellweger syndrome and rhizomelic chondrodysplasia punctata.
Pediatric research 1990;27(3):304-10.
1990: Hugo W Moser; Ann B Moser; W W Chen; Paul A Watkins
Adrenoleukodystrophy and Zellweger syndrome.
Progress in clinical and biological research 1990;321():511-35.
1989: Stephanie J Mihalik; Hugo W Moser; Paul A Watkins; D M Danks; A Poulos; W J Rhead
Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients.
Pediatric research 1989;25(5):548-52.
1989: Paul A Watkins; W W Chen; C J Harris; Gerald Hoefler; S Hoefler; D C Blake; A Balfe; Richard I Kelley; Ann B Moser; M E Beard
Peroxisomal bifunctional enzyme deficiency.
The Journal of clinical investigation 1989;83(3):771-7.
1989: Hugo W Moser; Stephanie J Mihalik; Paul A Watkins
Adrenoleukodystrophy and other peroxisomal disorders that affect the nervous system, including new observations on L-pipecolic acid oxidase in primates.
Brain & development 1989;11(2):80-90.
1988: S Hoefler; Gerald Hoefler; Ann B Moser; Paul A Watkins; W W Chen; Hugo W Moser
Prenatal diagnosis of rhizomelic chondrodysplasia punctata.
Prenatal diagnosis 1988;8(8):571-6.
1988: Sakkubai Naidu; Gerald Hoefler; Paul A Watkins; W W Chen; Ann B Moser; S Hoefler; N E Rance; J M Powers; M Beard; W Richard Green
Neonatal seizures and retardation in a girl with biochemical features of X-linked adrenoleukodystrophy: a possible new peroxisomal disease entity.
Neurology 1988;38(7):1100-7.
1988: Gerald Hoefler; S Hoefler; Paul A Watkins; W W Chen; Ann B Moser; V Baldwin; B McGillivary; J Charrow; J M Friedman; L Rutledge
Biochemical abnormalities in rhizomelic chondrodysplasia punctata.
The Journal of pediatrics 1988;112(5):726-33.
1987: Paul A Watkins; Y Kanaho; Joel Moss
Inhibition of the GTPase activity of transducin by an NAD+:arginine ADP-ribosyltransferase from turkey erythrocytes.
The Biochemical journal 1987;248(3):749-54.
1987: W W Chen; Paul A Watkins; T Osumi; T Hashimoto; H W Moser
Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy.
Proceedings of the National Academy of Sciences of the United States of America 1987;84(5):1425-8.
1987: Paul A Watkins; Sakkubai Naidu; H W Moser
Adrenoleukodystrophy: biochemical procedures in diagnosis, prevention and treatment.
Journal of inherited metabolic disease 1987;10 Suppl 1():46-53.
1986: Paul B LAZAROW; Y Fujiki; G M Small; Paul A Watkins; H Moser
Presence of the peroxisomal 22-kDa integral membrane protein in the liver of a person lacking recognizable peroxisomes (Zellweger syndrome).
Proceedings of the National Academy of Sciences of the United States of America 1986;83(23):9193-6.
1986: J Moss; S J Stanley; Paul A Watkins; Drusilla L Burns; C R Manclark; Harvey R Kaslow; E L Hewlett
Stimulation of the thiol-dependent ADP-ribosyltransferase and NAD glycohydrolase activities of Bordetella pertussis toxin by adenine nucleotides, phospholipids, and detergents.
Biochemistry 1986;25(9):2720-5.
1986: William B Rizzo; Paul A Watkins; M W Phillips; D Cranin; B Campbell; J Avigan
Adrenoleukodystrophy: oleic acid lowers fibroblast saturated C22-26 fatty acids.
Neurology 1986;36(3):357-61.
1985: Paul A Watkins; D L Burns; Y Kanaho; T Y Liu; E L Hewlett; J Moss
ADP-ribosylation of transducin by pertussis toxin.
The Journal of biological chemistry 1985;260(25):13478-82.
1985: Paul A Watkins; D A Yost; A W Chang; J J Mekalanos; J Moss
Detection of NAD:arginine ADPribosyltransferases in animal tissues using 125I-labeled 1-(p-hydroxyphenyl) 2-guanidinoethane as ADPribose acceptor.
Biochimica et biophysica acta 1985;840(3):401-8.
1984: J Moss; Paul A Watkins; S J Stanley; M R Purnell; W R Kidwell
Inactivation of glutamine synthetases by an NAD:arginine ADP-ribosyltransferase.
The Journal of biological chemistry 1984;259(8):5100-4.
1984: Paul A Watkins; J Moss; D L Burns; E L Hewlett; M Vaughan
Inhibition of bovine rod outer segment GTPase by Bordetella pertussis toxin.
The Journal of biological chemistry 1984;259(3):1378-81.
1984: Joel Moss; P Bruni; J A Hsia; S C Tsai; Paul A Watkins; JANE HALPERN; Drusilla L Burns; Y Kanaho; P P Chang; E L Hewlett
Pertussis toxin-catalyzed ADP-ribosylation: effects on the coupling of inhibitory receptors to the adenylate cyclase system.
Journal of receptor research 1984;4(1-6):459-74.
1983: Martha L Elks; Paul A Watkins; V C Manganiello; J Moss; E Hewlett; M Vaughan
Selective regulation by pertussis toxin of insulin-induced activation of particulate cAMP phosphodiesterase activity in 3T3-L1 adipocytes.
Biochemical and biophysical research communications 1983;116(2):593-8.
1983: J Moss; Paul A Watkins; D A Yost
Characterization of NAD: arginine ADP-ribosyltransferases in animal tissues.
Princess Takamatsu symposia 1983;13():103-9.
1982: Paul A Watkins; J Moss; P H Pekala; M D Lane
Effect of differentiation on the adenylate cyclase system of 3T3-C2 and 3T3-L1 cells. Determination of choleragen substrates in differentiating 3T3-L1 and nondifferentiating 3T3-C2 cells.
The Journal of biological chemistry 1982;257(24):14719-22.
1982: Paul A Watkins; J Moss
Effects of nucleotides on activity of a purified ADP-ribosyltransferase from turkey erythrocytes.
Archives of biochemistry and biophysics 1982;216(1):74-80.
1981: S Nakaya; Paul A Watkins; A J Bitonti; Leonard M Hjelmeland; J Moss; M Vaughan
GTP stabilization of adenylate cyclase activated and ADP-ribosylated by choleragen.
Biochemical and biophysical research communications 1981;102(1):66-74.
1981: Paul A Watkins; J Moss; M Vaughan
ADP ribosylation of membrane proteins from human fibroblasts. Effect of prior exposure of cells to choleragen.
The Journal of biological chemistry 1981;256(10):4895-9.
1981: P H Pekala; M D Lane; Paul A Watkins; J Moss
On the mechanism of preadipocyte differentiation. Masking of poly(ADP-ribose) synthetase activity during differentiation of 3T3-L1 preadipocytes.
The Journal of biological chemistry 1981;256(10):4871-6.
1980: J Moss; S J Stanley; Paul A Watkins; M Vaughan
ADP-ribosyltransferase activity of mono- and multi-(ADP-ribosylated) choleragen.
The Journal of biological chemistry 1980;255(16):7835-7.
1980: J Moss; S J Stanley; Paul A Watkins
Isolation and properties of an NAD- and guanidine-dependent ADP-ribosyltransferase from turkey erythrocytes.
The Journal of biological chemistry 1980;255(12):5838-40.
1980: Paul A Watkins; J Moss; M Vaughan
Effects of GTP on choleragen-catalyzed ADP ribosylation of membrane and soluble proteins.
The Journal of biological chemistry 1980;255(9):3959-63.
1979: M D Lane; Paul A Watkins; M J Meredith
Hormonal regulation of acetyl-CoA carboxylase activity in the liver cell.
CRC critical reviews in biochemistry 1979;7(2):121-41.
1979: S D Clarke; Paul A Watkins; M D Lane
Acute control of fatty acid synthesis by cyclic AMP in the chick liver cell: possible site of inhibition of citrate formation.
Journal of lipid research 1979;20(8):974-85.
1977: D M Tarlow; Paul A Watkins; R E Reed; R S Miller; E E Zwergel; M D Lane
Lipogenesis and the synthesis and secretion of very low density lipoprotein by avian liver cells in nonproliferating monolayer culture. Hormonal effects.
The Journal of cell biology 1977;73(2):332-53.
1977: Paul A Watkins; D M Tarlow; M D Lane
Mechanism for acute control of fatty acid synthesis by glucagon and 3':5'-cyclic AMP in the liver cell.
Proceedings of the National Academy of Sciences of the United States of America 1977;74(4):1497-501.