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

Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian... 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 (M r 35K) that restores the biogenesis of peroxisomes and complements the defect of peroxisomal functions in the mutant Z65. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Nature Springer Journals

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

Tsukamoto, Toshiro; Miura, Satoshi; Fujiki, Yukio
Nature , Volume 350 (6313) – Mar 7, 1991
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References (28)

Publisher
Springer Journals
Copyright
Copyright © 1991 by Nature Publishing Group
Subject
Science, Humanities and Social Sciences, multidisciplinary; Science, Humanities and Social Sciences, multidisciplinary; Science, multidisciplinary
ISSN
0028-0836
eISSN
1476-4687
DOI
10.1038/350077a0
Publisher site
See Article on Publisher Site

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 (M r 35K) that restores the biogenesis of peroxisomes and complements the defect of peroxisomal functions in the mutant Z65.

Journal

Nature – Springer Journals

Published: Mar 7, 1991

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