Targeted Disruption of the Peroxisomal Fatty Acyl-CoA Oxidase Gene: Generation of a Mouse Model of Pseudoneonatal Adrenoleukodystrophy (original ) (raw )A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy)
C. Roermund
American journal of human genetics, 1988
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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism
P Van Veldhoven
The Journal of Lipid Research, 2010
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Peroxisomal Very Long Chain Fatty Acid β-Oxidation Activity Is Determined by the Level of Adrenodeukodystrophy Protein (ALDP) Expression
L. Braiterman
Molecular Genetics and Metabolism, 1999
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Peroxisomal Fatty Acid β-Oxidation in Relation to Adrenoleukodystrophy
C. Roermund
Developmental Neuroscience, 1991
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Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids
C. Roermund
Biochemical and Biophysical Research Communications, 1988
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Targeted Disruption of the Peroxisomal Fatty Acyl-CoA Oxidase Gene: Generation of a Mouse Model of Pseudoneonatal Adrenoleukodystrophya
nobuteru usuda
Annals of the New …, 1996
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Hepatocellular and Hepatic Peroxisomal Alterations In Mice With a Disrupted Peroxisomal Fatty Acyl-Coenzyme A Oxidase Gene
nobuteru usuda
Journal of Biological …, 1996
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Journal of Inherited Metabolic Disease, 1990
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Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2006
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Biochemical Society Transactions, 2001
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Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders
Manuel Martinez-Rivera
Brain Research, 1992
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Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy
Hugo Heymans
Proceedings of the National Academy of Sciences, 1987
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Peroxisome mosaicism in the livers of peroxisomal deficiency patients*1
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Hepatology, 1995
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Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders
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Journal of Clinical Investigation, 1987
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Plasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls
Sakkubai Naidu
Annals of Neurology, 1999
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The Role of the Carnitine System in Peroxisomal Fatty Acid Oxidation
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The American Journal of the Medical Sciences, 1999
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Clinical and Biochemical Pitfalls in the Diagnosis of Peroxisomal Disorders
Ronald Wanders
Neuropediatrics, 2016
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Lactic acidosis and mitochondrial dysfunction in two children with peroxisomal disorders
Panagiota Tsatsos
Journal of Inherited Metabolic Disease, 1993
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Metabolism of saturated and polyunsaturated very-long-chain fatty acids in fibroblasts from patients with defects in peroxisomal β-oxidation
Jackie Street
Biochemical Journal, 1990
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Peroxisomal disorders I: biochemistry and genetics of peroxisome biogenesis disorders
Ronald Wanders
Clinical Genetics, 2004
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A new type of peroxisomal disorder with variable expression in liver and fibroblasts
Moshe Berant , Theodore Iancu , Frank Roels
The Journal of Pediatrics, 1994
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Peroxisomal D-hydroxyacyl-CoA Dehydrogenase Deficiency: Resolution of the Enzyme Defect and Its Molecular Basis in Bifunctional Protein Deficiency
Dean Cuebas
Nucleic Acids Research, 1998
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Peroxisome biogenesis disorders with prolonged survival: Phenotypic expression in a cohort of 31 patients
Ronald Wanders
American Journal of Medical Genetics, 2004
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Peroxisomes, lipid metabolism, and peroxisomal disorders
Ronald Wanders
Molecular Genetics and Metabolism, 2004
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