Peroxisomal membrane ghosts in Zellweger syndrome--aberrant organelle assembly

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Science  25 Mar 1988:
Vol. 239, Issue 4847, pp. 1536-1538
DOI: 10.1126/science.3281254


Peroxisomes are apparently missing in Zellweger syndrome; nevertheless, some of the integral membrane proteins of the organelle are present. Their distribution was studied by immunofluorescence microscopy. In control fibroblasts, peroxisomes appeared as small dots. In Zellweger fibroblasts, the peroxisomal membrane proteins were located in unusual empty membrane structures of larger size. These results suggest that the primary defect in this disease may be in the mechanism for import of matrix proteins.

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