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Science 25 March 1988:
Vol. 239. no. 4847, pp. 1536 - 1538
DOI: 10.1126/science.3281254
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Articles

Science, Vol 239, Issue 4847, 1536-1538
Copyright © 1988 by American Association for the Advancement of Science

articles

Peroxisomal membrane ghosts in Zellweger syndrome--aberrant organelle assembly

MJ Santos, T Imanaka, H Shio, GM Small, and PB Lazarow

Rockefeller University, New York, NY 10021.

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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