Histochemistry of peroxisomal enzyme activities: a tool in the diagnosis of Zellweger syndrome.
The localization of the activity of the peroxisomal enzymes D-amino acid oxidase and hydroxyacid oxidase was studied at the light-microscopical level in livers and kidneys of control subjects and patients with Zellweger syndrome, an inherited disease characterized by a lack of intact peroxisomes. D-Amino acid oxidase and hydroxyacid oxidase activities were demonstrated in unfixed cryostat sections with the cerium-diaminobenzidine-cobalt-hydrogen peroxide procedure, in which cerium ions capture hydrogen peroxide, the product of both enzymes. In a second step reaction decomposition of cerium perhydroxide gives rise to a diaminobenzidine polymer complexed with cobalt ions. D-Amino acid oxidase and hydroxyacid oxidase activities were found in peroxisomes of liver parenchymal cells, and only D-amino acid oxidase in peroxisomes of proximal tubular cells of kidneys of control humans. The activities of these enzymes were not detectable in livers and kidneys of Zellweger patients. It is concluded that the cerium-diaminobenzidine-cobalt-hydrogen peroxide procedure enables the demonstration of peroxisomal enzyme activities in human tissues at the light-microscopical level and is an important tool in detecting patients with Zellweger syndrome.
Frederiks
W M
WM
Laboratory of Cell Biology and Histology, University of Amsterdam, The Netherlands.
Bosch
K S
KS
Ankum
M
M
Wanders
R J
RJ
D000429:Alcohol Oxidoreductases / Q000032:analysis; D003605:D-Amino-Acid Oxidase / Q000032:analysis; D006651:Histocytochemistry; D006801:Humans; D007668:Kidney / Q000201:enzymology / Q000473:pathology; D008099:Liver / Q000201:enzymology / Q000473:pathology; D008830:Microbodies / Q000201:enzymology*; D015211:Zellweger Syndrome / Q000175:diagnosis* / Q000201:enzymology* / Q000473:pathology
D000429:Alcohol Oxidoreductases; D003605:D-Amino-Acid Oxidase