Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant

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Abstract

PEROXISOMES are among the intracellular organdies of eukaryotic cells that contain specialized sets of enzymes with specific functions1. Little is known of membranous components involved in assembly of the intracellular compartments2-5. We isolated two peroxisome-deficient and mutually complementary, Chinese hamster ovary cell mutants, Z65 and Z246, which closely resembled fibroblasts from patients with autosomal recessive, peroxisome-defective disorders such as Zellweger syndrome1,7. These patients show characteristic dysmorphism, severe hypotonia, psychomotor retardation, and peroxisomal dysfunctions and rarely survive early childhood. Here we report what seems to be the first direct cloning and characterization of a complementary DNA encoding a peroxisomal membrane protein of relative molecular mass 35,000 (Mr 35K) that restores the biogenesis of peroxisomes and complements the defect of peroxisomal functions in the mutant Z65.

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Tsukamoto, T., Miura, S. & Fujiki, Y. Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant. Nature 350, 77–81 (1991) doi:10.1038/350077a0

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