Journal
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
Volume 1763, Issue 12, Pages 1374-1381Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbamcr.2006.09.012
Keywords
CHO cell mutant; genetic phenotype-complementation; peroxin; peroxisome ghost; Zellweger syndrome; patients' fibroblast; pathogenic gene
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Cells with a genetic defect affecting a biological activity and/or a cell phenotype are generally called cell mutants and are a highly useful tool in genetic, biochemical, as well as cell biological research. To investigate peroxisome biogenesis and human peroxisome biogenesis disorders, more than a dozen complementation groups of Chinese hamster ovary (CHO) cell mutants defective in peroxisome assembly have been successfully isolated and established as a model system. Moreover, successful PEXgene cloning studies by taking advantage of rapid functional complementation assay of CHO cell mutants invaluably contributed to the accomplishment of isolation of pathogenic genes responsible for peroxisome biogenesis diseases. Molecular mechanisms of peroxisome assembly are currently investigated by making use of such mammalian cell mutants. (c) 2006 Elsevier B.V. All rights reserved.
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