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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The inborn errors of peroxisomal β-oxidation: A review

Hugo Heymans

Journal of Inherited Metabolic Disease, 1990

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Peroxisomal disorders: The single peroxisomal enzyme deficiencies

Ronald Wanders

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2006

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Peroxisomal fatty acid α- and β-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases

Ronald Wanders

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

Marc Espeel, ingrid kerckaert

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

Carlo van Roermund

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

Rona Ramsay

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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