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Article

Peroxisomal Impairment in Niemann-Pick Type C Disease

1 Mar 1997

Abstract excerpt

Niemann-Pick type C disease (NPC) belongs to the group of lysosomal storage diseases characterized by an accumulation of cholesterol and sphingomyelin. Using a mutant mouse strain, enzymatic markers for lysosomes, mitochondria, microsomes, and peroxisomes were investigated in the liver and brain. Aside from lysosomal changes, we found a sizable decrease of peroxisomal beta-oxidation of fatty acids and catalase...

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PMID: 9045641
DOI: 10.1074/jbc.272.10.6245

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