Overall there is a considerable increase in the association of catalase mRNA with polysomes in ZS cell lines as compared to control indicating translational upregulation.
Although plasma very-long-chain fatty acid profile suggested X-linked adrenoleukodystrophy, marked reduction in fibroblast lignoceric acid oxidation and the presence of cytosolic catalase were consistent with Zellweger syndrome (ZS).
Cell fusion and immunofluorescence analyses of peroxisome size and catalase packaging formally demonstrate genetic complementation groups for peroxisome assembly in Zellweger syndrome.
Catalase was found to sediment with the peroxisomes in the homogenates of normal cells, but to remain in the supernatant with Zellweger syndrome amniocyte homogenates.