Peroxisome biogenesis and molecular defects in peroxisome assembly disorders

Cell Biochemistry and Biophysics
Y FujikiS Tamura

Abstract

Peroxisome assembly in mammals requires more than 14 genes. So far, we have isolated seven complementation groups (CGs) of peroxisome biogenesis-defective Chinese hamster ovary (CHO) cell mutants, Z65, Z24/ZP107, ZP92, ZP105/ZP139, ZP109, ZP110, ZP114. Two peroxin cDNAs, PEX2 and PEX6, were first cloned by genetic phenotype-complementation assay using Z65 and ZP92, respectively, and were shown to be responsible for peroxisome biogenesis disorders (PBD) such as Zellweger syndrome, of CG-F (the same as CG-X in U.S.A.) and CG-C (the same as CG-IV), respectively. Pex2p is a RING zinc finger membrane protein of peroxisomes and Pex6p is a member of the AAA ATPase family. We likewise isolated PEX12 encoding a peroxisomal integral membrane protein in the RING family, by functional complementation of ZP109, demonstrating PEX12 to be responsible for CG-III PBD. We also cloned PEX1 by screening of human liver cDNA library, using ZP107. PEX1 mutation was delineated to be the genetic cause of PBD in the most highest incidence group, CG-E (the same as CG-I). Moreover, we recently found that Pex5p is involved in transport of not only PTS1- but also PTS2-protein, distinct from yeast Pex5p, using PEX5-defective ZP105 and ZP139. Thus, CHO cell m...Continue Reading

Citations

Feb 22, 2003·Journal of Molecular Biology·José R PiresRalf Erdmann
Mar 6, 2004·Seminars in Neonatology : SN·Eve A Roberts
Jan 7, 2005·The FEBS Journal·Ingvild BirschmannWolf-H Kunau
Mar 13, 2014·Neurochemistry International·Denis I Crane
Jun 11, 2002·Biochemical and Biophysical Research Communications·Eiko YanagoYukio Fujiki
Aug 26, 2004·The Journal of Biological Chemistry·Kumiko ShiozawaHidekazu Hiroaki

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