Immunochemical analysis of the peroxisomal beta-oxidation enzymes in rat and human heart and skeletal muscle and in skeletal muscle of Zellweger patients.
Immunoblot analyses with antibodies against the peroxisomal beta-oxidation enzymes from rat liver showed the presence of these enzymes in rat and human liver and kidney and rat adrenal gland. The bifunctional protein could not be detected in muscle tissues or cultured muscle cells. Acyl-CoA oxidase was detected in rat heart and cultured human muscle cells. 3-Ketoacyl-CoA thiolase was also detected in human and rat heart and skeletal muscle; however, this enzyme was not detectable in skeletal muscle of Zellweger patients, in agreement with the absence of peroxisomal fatty acid oxidation.
Reubsaet
F A
FA
Department of Pediatrics, University of Nijmegen, The Netherlands.
Brückwilder
M L
ML
Veerkamp
J H
JH
Trijbels
J M
JM
Hashimoto
T
T
Monnens
L A
LA
D000102:Acetyl-CoA C-Acyltransferase / Q000302:isolation & purification / Q000378:metabolism*; D000311:Adrenal Glands / Q000201:enzymology; D000818:Animals; D002478:Cells, Cultured; D004591:Electrophoresis, Polyacrylamide Gel; D044943:Fatty Acid Desaturases / Q000378:metabolism*; D006801:Humans; D015151:Immunoblotting; D007668:Kidney / Q000201:enzymology; D008297:Male; D008830:Microbodies / Q000201:enzymology*; D008970:Molecular Weight; D009132:Muscles / Q000201:enzymology*; D009206:Myocardium / Q000201:enzymology*; D051381:Rats; D011919:Rats, Inbred Strains; D012016:Reference Values; D015211:Zellweger Syndrome / Q000201:enzymology*
D044943:Fatty Acid Desaturases; D000102:Acetyl-CoA C-Acyltransferase