Newly identified Chinese hamster ovary cell mutants defective in peroxisome assembly represent complementation group A of human peroxisome biogenesis disorders and one novel group in mammals

被引:21
作者
Ghaedi, K
Itagaki, A
Toyama, R
Tamura, S
Matsumura, T
Kawai, A
Shimozawa, N
Suzuki, Y
Kondo, N
Fujiki, Y
机构
[1] Kyushu Univ, Fac Sci, Dept Biol, Higashi Ku, Fukuoka 8128581, Japan
[2] Gifu Univ, Dept Pediat, Gifu 5008076, Japan
[3] Japan Sci & Technol Corp, CREST, Tokyo 1700013, Japan
关键词
CHO cell mutants; peroxisome biogenesis; complementation groups; Zellweger syndrome; peroxin;
D O I
10.1006/excr.1999.4412
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
We isolated peroxisome biogenesis-defective mutants from rat PEX2-transformed Chinese hamster ovary (CHO) cells, using the 9-(1'-pyrene)nonanol/ultraviolet method. A total of 18 mutant cell clones showing cytosolic localization of catalase were isolated. By complementation group (CG;) analysis by means of PEX cDNA transfection and cell fusion, cell mutants, ZP124 and ZP126, were found to belong to two novel CGs of CHO mutants. Mutants, ZP135 and ZP167, mere also classified to the same CG as ZP124. Further cell fusion analysis using 12 CGs fibroblasts from patients with peroxisome deficiency disorders such as Zellweger syndrome revealed that ZP124 belonged to human CG-A, the same group as CC-WI in the United States. ZP126 could not be classified to any of human and CHO CGs. These mutants also showed typical peroxisome assembly-defective phenotypes such as severe loss of catalase latency and impaired biogenesis of peroxisomal enzymes. Collectively, ZP124 represents CG-A, and ZP126 is in a newly identified CG distinct from the 14 mammalian CGs previously characterized, (C) 1999 Academic Press.
引用
收藏
页码:482 / 488
页数:7
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