X-Linked adrenoleukodystrophy: Defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters

Peroxisomal and Mitochondrial Defects in the Cerebro-Hepato-Renal Syndrome

1973 • 777 citations

Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.

1984 • 344 citations

Peroxisomal disorders: A newly recognised group of genetic diseases

1986 • 313 citations

Adrenoleukodystrophy: Survey of 303 cases: Biochemistry, diagnosis, and therapy

1984 • 256 citations

Purification and Properties of Acyl-CoA Oxidase from Rat Liver1

1980 • 252 citations

Neonatal adrenoleukodystrophy: New cases, biochemical studies, and differentiation from Zellweger and related peroxisomal polydystrophy syndromes

1986 • 216 citations

Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts

1980 • 199 citations

Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency.

1987 • 198 citations

A New Method for the Preparation of Acyl-CoA Thioesters1

1981 • 169 citations

Lignoceroyl‐CoASH ligase: enzyme defect in fatty acid β‐oxidation system in X‐linked childhood adrenoleukodystrophy

1986 • 165 citations

Peroxisomal Defects in Neonatal-Onset and X-Linked Adrenoleukodystrophies

1985 • 150 citations

Properties of Mitochondrial and Peroxisomal Enoyl-CoA Hydratases from Rat Liver1

1980 • 150 citations

Bronzekrankheit und sklerosierende Encephalomyelitis

1923 • 143 citations

Adrenoleukodystrophy: Impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex

1981 • 130 citations

Peroxisomal β-oxidation enzyme proteins in the Zellweger syndrome

1985 • 128 citations

Adreno-leukodystrophy (adreno-testiculo-leukomyelo-neuropathic-complex).

1985 • 108 citations

Hepatic peroxisomes are deficient in infantile refsum disease: A cytochemical study of 4 cases

1986 • 101 citations

Properties of Peroxisomal 3-Ketoacyl-CoA Thiolase from Rat Liver1

1981 • 98 citations

C27 Bile Acids in Infants with Coprostanic Acidemia and Occurrence of a 3alpha,7alpha,12alpha-Trihydroxy-5beta-C29 Dicarboxylic Bile Acid as a Major Component in Their Serum

1979 • 90 citations

Zellwegar Syndrome: Biochemical and Morphological Studies on Two Patients Treated with Clofibrate

1985 • 84 citations

High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients

1978 • 81 citations

Adreno-leukodystrophy (adreno-testiculo-leukomyelo-neuropathic-complex).

1985 • 76 citations

Synthesis of 3-ketoacyl-CoA thiolase of rat liver peroxisomes on free polyribosomes as a larger precursor. Induction of thiolase mRNA activity by clofibrate

1985 • 73 citations

Peroxisomal very long-chain fatty acid β-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders

1987 • 56 citations

A comparative study of stearic and lignoceric acid oxidation by human skin fibroblasts

1986 • 47 citations

Studies on the peroxisomal oxidation of palmitate and lignocerate in rat liver

1987 • 43 citations

Deficient activities and proteins of peroxisomal β-oxidation enzymes in infants with Zellweger syndrome

1986 • 42 citations

Proteolytic digestion patterns of spectrin subunits

1978 • 32 citations

Peroxisomal fatty acid β‐oxidation in human skin fibroblasts: X‐linked adrenoleukodystrophy, a peroxisomal very long chain fatty acyl‐CoA synthetase deficiency?

1987 • 12 citations