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Results: 1-25 | 26-44
Results: 1-25/44
GENE REDUNDANCY AND PHARMACOLOGICAL GENE-THERAPY - IMPLICATIONS FOR X-LINKED ADRENOLEUKODYSTROPHY
Authors: KEMP S WEI HM LU JF BRAITERMAN LT MCGUINNESS MC MOSER AB WATKINS PA SMITH KD
Citation: S. Kemp et al., GENE REDUNDANCY AND PHARMACOLOGICAL GENE-THERAPY - IMPLICATIONS FOR X-LINKED ADRENOLEUKODYSTROPHY, Nature medicine, 4(11), 1998, pp. 1261-1268
SUPPRESSION OF PEROXISOMAL MEMBRANE-PROTEIN DEFECTS BY PEROXISOMAL ATP BINDING CASSETTE (ABC) PROTEINS
Authors: BRAITERMAN LT ZHENG SQ WATKINS PA GERAGHTY MT JOHNSON G MCGUINNESS MC MOSER AB SMITH KD
Citation: Lt. Braiterman et al., SUPPRESSION OF PEROXISOMAL MEMBRANE-PROTEIN DEFECTS BY PEROXISOMAL ATP BINDING CASSETTE (ABC) PROTEINS, Human molecular genetics, 7(2), 1998, pp. 239-247
RESTORATION OF PEX2 PEROXISOME ASSEMBLY DEFECTS BY OVEREXPRESSION OF PMP70
Authors: GARTNER J BROSIUS U OBIE C WATKINS PA VALLE D
Citation: J. Gartner et al., RESTORATION OF PEX2 PEROXISOME ASSEMBLY DEFECTS BY OVEREXPRESSION OF PMP70, European journal of cell biology, 76(4), 1998, pp. 237-245
THE EFFECT OF CHRONIC LUTEINIZING-HORMONE TREATMENT ON ADULT-RAT LEYDIG-CELLS
Authors: MENDISHANDAGAMA SMLC WATKINS PA GELBER SJ SCALLEN TJ
Citation: Smlc. Mendishandagama et al., THE EFFECT OF CHRONIC LUTEINIZING-HORMONE TREATMENT ON ADULT-RAT LEYDIG-CELLS, Tissue & cell, 30(1), 1998, pp. 64-73
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
Authors: WATKINS PA LU JF STEINBERG SJ GOULD SJ SMITH KD BRAITERMAN LT
Citation: Pa. Watkins et al., 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, 273(29), 1998, pp. 18210-18219
CHARACTERIZATION OF PHYTANOYL COENZYME-A HYDROXYLASE IN HUMAN LIVER AND ACTIVITY MEASUREMENTS IN PATIENTS WITH PEROXISOMAL DISORDERS
Authors: JANSEN GA MIHALIK SJ WATKINS PA JAKOBS C MOSER HW WANDERS RJA
Citation: Ga. Jansen et al., CHARACTERIZATION OF PHYTANOYL COENZYME-A HYDROXYLASE IN HUMAN LIVER AND ACTIVITY MEASUREMENTS IN PATIENTS WITH PEROXISOMAL DISORDERS, Clinica chimica acta, 271(2), 1998, pp. 203-211
IDENTIFICATION OF PAHX, A REFSUM-DISEASE GENE
Authors: MIHALIK SJ MORRELL JC KIM D SACKSTEDER KA WATKINS PA GOULD SJ
Citation: Sj. Mihalik et al., IDENTIFICATION OF PAHX, A REFSUM-DISEASE GENE, Nature genetics, 17(2), 1997, pp. 185-189
THE PRODUCT OF THE SACCHAROMYCES-CEREVISIAE FAT1 LOCUS HAS PEROXISOMAL VERY LONG-CHAIN ACYL-COA SYNTHETASE (VLCS) ACTIVITY
Authors: WATKINS PA BRAITERMAN LT LU JF STEINBERG SJ SHANI N SMITH KD
Citation: Pa. Watkins et al., THE PRODUCT OF THE SACCHAROMYCES-CEREVISIAE FAT1 LOCUS HAS PEROXISOMAL VERY LONG-CHAIN ACYL-COA SYNTHETASE (VLCS) ACTIVITY, Molecular biology of the cell, 8, 1997, pp. 667-667
STUDIES OF THE X-LINKED ADRENOLEUKODYSTROPHY PROTEIN (ALDP) AND SUPPRESSION OF PEROXISOME BIOGENESIS MEMBRANE-PROTEIN DEFECTS
Authors: BRAITERMAN LT ZHENG S WATKINS PA GERAGHTY M JOHNSON G MCGUINNESS MC MOSER AB SMITH KD
Citation: Lt. Braiterman et al., STUDIES OF THE X-LINKED ADRENOLEUKODYSTROPHY PROTEIN (ALDP) AND SUPPRESSION OF PEROXISOME BIOGENESIS MEMBRANE-PROTEIN DEFECTS, Molecular biology of the cell, 8, 1997, pp. 670-670
X-CHROMOSOME INACTIVATION IN SYMPTOMATIC HETEROZYGOTES OF X-LINKED ADRENOLEUKODYSTROPHY
Authors: NAIDU S WASHINGTON C THIRUMALAI S SMITH KD MOSER HW WATKINS PA
Citation: S. Naidu et al., X-CHROMOSOME INACTIVATION IN SYMPTOMATIC HETEROZYGOTES OF X-LINKED ADRENOLEUKODYSTROPHY, Annals of neurology, 42(3), 1997, pp. 33-33
FATTY-ACID ACTIVATION
Authors: WATKINS PA
Citation: Pa. Watkins, FATTY-ACID ACTIVATION, Progress in lipid research, 36(1), 1997, pp. 55-83
PHYTANOYL-COA HYDROXYLASE IS NOT ONLY DEFICIENT IN CLASSICAL REFSUM-DISEASE BUT ALSO IN RHIZOMELIC CHONDRODYSPLASIA PUNCTATA
Authors: JANSEN GA MIHALIK SJ WATKINS PA MOSER HW JAKOBS C HEIJMANS HSA WANDERS RJA
Citation: Ga. Jansen et al., PHYTANOYL-COA HYDROXYLASE IS NOT ONLY DEFICIENT IN CLASSICAL REFSUM-DISEASE BUT ALSO IN RHIZOMELIC CHONDRODYSPLASIA PUNCTATA, Journal of inherited metabolic disease, 20(3), 1997, pp. 444-446
PHYTANOYL-COENZYME-A HYDROXYLASE DEFICIENCY - THE ENZYME DEFECT IN REFSUMS-DISEASE
Authors: JANSEN GA WANDERS RJA WATKINS PA MIHALIK SJ
Citation: Ga. Jansen et al., PHYTANOYL-COENZYME-A HYDROXYLASE DEFICIENCY - THE ENZYME DEFECT IN REFSUMS-DISEASE, The New England journal of medicine, 337(2), 1997, pp. 133-134
A MOUSE MODEL FOR X-LINKED ADRENOLEUKODYSTROPHY
Authors: LU JF LAWLER AM WATKINS PA POWERS JM MOSER AB MOSER HW SMITH KD
Citation: Jf. Lu et al., A MOUSE MODEL FOR X-LINKED ADRENOLEUKODYSTROPHY, Proceedings of the National Academy of Sciences of the United Statesof America, 94(17), 1997, pp. 9366-9371
PAHX IS A NEWLY IDENTIFIED GENE WHICH CODES FOR THE PEROXISOMAL PROTEIN PHYTANOYL-COA ALPHA-HYDROXYLASE AND IS DEFECTIVE IN REFSUM-DISEASE
Authors: MIHALIK SJ MORRELL JC KIM DG WATKINS PA GOULD SJ
Citation: Sj. Mihalik et al., PAHX IS A NEWLY IDENTIFIED GENE WHICH CODES FOR THE PEROXISOMAL PROTEIN PHYTANOYL-COA ALPHA-HYDROXYLASE AND IS DEFECTIVE IN REFSUM-DISEASE, American journal of human genetics, 61(4), 1997, pp. 53-53
CHARACTERIZATION OF AN X-LINKED ADRENOLEUKODYSTROPHY MOUSE MODEL
Authors: LU JF LAWLER AM WATKINS PA POWERS JM KEMP B MORAN TH MOSER AB MOSER HW SMITH KD
Citation: Jf. Lu et al., CHARACTERIZATION OF AN X-LINKED ADRENOLEUKODYSTROPHY MOUSE MODEL, American journal of human genetics, 61(4), 1997, pp. 285-285
STUDIES OF THE X-LINKED ADRENOLEUKODYSTROPHY PROTEIN (ALDP) AND SUPPRESSION OF PEROXISOME BIOGENESIS MEMBRANE-PROTEIN DEFECTS
Authors: BRAITERMAN LT ZHENG S WATKINS PA GERAGHTY M JOHNSON G MCGUINNESS MC MOSER AB SMITH KD
Citation: Lt. Braiterman et al., STUDIES OF THE X-LINKED ADRENOLEUKODYSTROPHY PROTEIN (ALDP) AND SUPPRESSION OF PEROXISOME BIOGENESIS MEMBRANE-PROTEIN DEFECTS, American journal of human genetics, 61(4), 1997, pp. 1447-1447
PHARMACOLOGICAL RESCUE OF THE X-LINKED ADRENOLEUKODYSTROPHY BIOCHEMICAL-ABNORMALITY, IN-VITRO
Authors: KEMP S WEI HM LU JF WATKINS PA SMITH KD
Citation: S. Kemp et al., PHARMACOLOGICAL RESCUE OF THE X-LINKED ADRENOLEUKODYSTROPHY BIOCHEMICAL-ABNORMALITY, IN-VITRO, American journal of human genetics, 61(4), 1997, pp. 1478-1478
VERY LONG-CHAIN FATTY-ACID BETA-OXIDATION ACTIVITY IS DETERMINED BY LEVEL OF ADRENOLEUKODYSTROPHY PROTEIN (ALDP) EXPRESSION
Authors: SMITH KD BRAITERMAN LT WATKINS PA
Citation: Kd. Smith et al., VERY LONG-CHAIN FATTY-ACID BETA-OXIDATION ACTIVITY IS DETERMINED BY LEVEL OF ADRENOLEUKODYSTROPHY PROTEIN (ALDP) EXPRESSION, American journal of human genetics, 61(4), 1997, pp. 1521-1521
CLONING A PUTATIVE HUMAN PEROXISOMAL VERY LONG-CHAIN ACYL COA SYNTHETASE (VLCS) PROTEIN TO FURTHER UNDERSTAND THE BIOCHEMICAL-DEFICIENCY INADRENOLEUKODYSTROPHY (ALD)
Authors: STEINBERG SJ KEMP S BRAITERMAN L WATKINS PA
Citation: Sj. Steinberg et al., CLONING A PUTATIVE HUMAN PEROXISOMAL VERY LONG-CHAIN ACYL COA SYNTHETASE (VLCS) PROTEIN TO FURTHER UNDERSTAND THE BIOCHEMICAL-DEFICIENCY INADRENOLEUKODYSTROPHY (ALD), American journal of human genetics, 61(4), 1997, pp. 1523-1523
DISRUPTION OF A YEAST VERY LONG-CHAIN ACYL-COA SYNTHETASE (VLCS) GENESIMULATES THE CELLULAR PHENOTYPE OF X-LINKED ADRENOLEUKODYSTROPHY (ALD)
Authors: WATKINS PA BRAITERMAN LT LU JF STEINBERG SJ SHANI N SMITH KD
Citation: Pa. Watkins et al., DISRUPTION OF A YEAST VERY LONG-CHAIN ACYL-COA SYNTHETASE (VLCS) GENESIMULATES THE CELLULAR PHENOTYPE OF X-LINKED ADRENOLEUKODYSTROPHY (ALD), American journal of human genetics, 61(4), 1997, pp. 1533-1533
NEURONAL MIGRATION ABNORMALITY IN PEROXISOMAL BIFUNCTIONAL ENZYME DEFECT
Authors: KAUFMANN WE THEDA C NAIDU S WATKINS PA MOSER AB MOSER HW
Citation: We. Kaufmann et al., NEURONAL MIGRATION ABNORMALITY IN PEROXISOMAL BIFUNCTIONAL ENZYME DEFECT, Annals of neurology, 39(2), 1996, pp. 268-271
PHYTANIC ACID ACTIVATION IN RAT-LIVER PEROXISOMES IS CATALYZED BY LONG-CHAIN ACYL-COA SYNTHETASE
Authors: WATKINS PA HOWARD AE GOULD SJ AVIGAN J MIHALIK SJ
Citation: Pa. Watkins et al., PHYTANIC ACID ACTIVATION IN RAT-LIVER PEROXISOMES IS CATALYZED BY LONG-CHAIN ACYL-COA SYNTHETASE, Journal of lipid research, 37(11), 1996, pp. 2288-2295
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
Authors: JANSEN GA MIHALIK SJ WATKINS PA MOSER HW JAKOBS C DENIS S WANDERS RJA
Citation: Ga. Jansen et al., 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, 229(1), 1996, pp. 205-210
MUTATIONAL ANALYSIS OF PATIENTS WITH X-LINKED ADRENOLEUKODYSTROPHY
Authors: KOK F NEUMANN S SARDE CO ZHENG S WU KH WEI HM BERGIN J WATKINS PA GOULD S SACK G MOSER H MANDEL JL SMITH KD
Citation: F. Kok et al., MUTATIONAL ANALYSIS OF PATIENTS WITH X-LINKED ADRENOLEUKODYSTROPHY, Human mutation, 6(2), 1995, pp. 104-115
Risultati: 1-25 | 26-44