Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency.

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

Purification and Properties of Acyl-CoA Oxidase from Rat Liver1

1980 • 252 citations

Activity of peroxisomal enzymes and intracellular distribution of catalase in Zellweger syndrome

1984 • 200 citations

Deficiency of acyl-CoA: Dihydroxyacetone phosphate acyltransferase in patients with Zellweger (cerebro-hepato-renal) syndrome

1984 • 192 citations

Pseudo-Zellweger syndrome: Deficiencies in several peroxisomal oxidative activities

1986 • 191 citations

Deficiency of Enzymes Catalyzing the Biosynthesis of Glycerol-Ether Lipids in Zellweger Syndrome

1984 • 186 citations

Peroxisomal βoxidation system of rat liver. Copurification of enoyl-CoA hydratase and 3-hydroxyacyl-CoA dehydrogenase

1979 • 185 citations

The cerebrohepatorenal syndrome of Zellweger, morphologic and metabolic aspects

1983 • 183 citations

Formation of cholic acid from 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid by rat liver peroxisomes.

1983 • 164 citations

Peroxisomal β-oxidation enzyme proteins in the Zellweger syndrome

1985 • 128 citations

Biosynthesis and intracellular transport of enzymes of peroxisomal beta-oxidation.

1984 • 124 citations

Peroxisomes (microbodies) in cell pathology.

1984 • 97 citations

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

1985 • 84 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

Degradation of cholesterol to propionic acid by rat liver peroxisomes

1982 • 62 citations

Peroxisomal β-oxidation of palmitoyl-CoA in human liver homogenates and its deficiency in the cerebro-hepato-renal (Zellweger) syndrome

1986 • 51 citations