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

Kamran Ghaedi, Atsushi Itagaki, Ryusuke Toyama, Shigehiko Tamura, Tsuyoshi Matsumura, Atsushi Kawai, Nobuyuki Shimozawa, Yasuyuki Suzuki, Naomi Kondo, Yukio Fujiki

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Abstract

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, were 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 CG-VIII 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.

Original languageEnglish
Pages (from-to)482-488
Number of pages7
JournalExperimental Cell Research
Volume248
Issue number2
DOIs
Publication statusPublished - May 1 1999

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Peroxisomes
Cricetulus
Mammals
Ovary
Cell Fusion
Catalase
Zellweger Syndrome
Transfection
Complementary DNA
Clone Cells
Fibroblasts
Phenotype
Enzymes
Peroxisome biogenesis disorders

All Science Journal Classification (ASJC) codes

  • Cell Biology

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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. / Ghaedi, Kamran; Itagaki, Atsushi; Toyama, Ryusuke; Tamura, Shigehiko; Matsumura, Tsuyoshi; Kawai, Atsushi; Shimozawa, Nobuyuki; Suzuki, Yasuyuki; Kondo, Naomi; Fujiki, Yukio.

In: Experimental Cell Research, Vol. 248, No. 2, 01.05.1999, p. 482-488.

Research output: Contribution to journalArticle

Ghaedi, Kamran ; Itagaki, Atsushi ; Toyama, Ryusuke ; Tamura, Shigehiko ; Matsumura, Tsuyoshi ; Kawai, Atsushi ; Shimozawa, Nobuyuki ; Suzuki, Yasuyuki ; Kondo, Naomi ; Fujiki, Yukio. / 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. In: Experimental Cell Research. 1999 ; Vol. 248, No. 2. pp. 482-488.
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