Immunochemical and biochemical studies of fatty acid oxidation in fibroblasts of Zellweger and X-linked adrenoleukodystrophy patients.
Immunoblot analyses of peroxisomal beta-oxidation enzymes showed that subunit A of acyl-CoA oxidase gave a stronger immunoreaction in fibroblasts of Zellweger and X-linked adrenoleukodystrophy patients than in those of controls. Subunits B and C and 3-ketoacyl-CoA thiolase were detected in fibroblasts of controls and X-linked adrenoleukodystrophy patients, but not of Zellweger patients. Total oxidation of palmitic and lignoceric acid was normal in homogenates of fibroblasts from Zellweger and X-linked adrenoleukodystrophy patients. The peroxisomal oxidation of both acids was only deficient in Zellweger patients. These data may not reflect the situation in vivo, as is evident from the accumulation of very-long-chain fatty acids in Zellweger and X-linked adrenoleukodystrophy patients.
Reubsaet
F A
FA
Department of Pediatrics, University of Nijmegen, The Netherlands.
Veerkamp
J H
JH
Brückwilder
M L
ML
Trijbels
J M
JM
Hashimoto
T
T
Monnens
L A
LA
D000102:Acetyl-CoA C-Acyltransferase / Q000378:metabolism; D042962:Acyl-CoA Oxidase; D000326:Adrenoleukodystrophy / Q000097:blood / Q000235:genetics / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D015151:Immunoblotting; D008830:Microbodies / Q000378:metabolism; D010084:Oxidation-Reduction; D010088:Oxidoreductases / Q000378:metabolism; D019308:Palmitic Acid; D010169:Palmitic Acids / Q000378:metabolism; D014960:X Chromosome; D015211:Zellweger Syndrome / Q000097:blood / Q000378:metabolism*
D005227:Fatty Acids; D010169:Palmitic Acids; D019308:Palmitic Acid; D010088:Oxidoreductases; D042962:Acyl-CoA Oxidase; D000102:Acetyl-CoA C-Acyltransferase; C010210:lignoceric acid