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J. Biol. Chem., Vol. 275, Issue 47, 37271-37277, November 24, 2000

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Catalase-less Peroxisomes
IMPLICATION IN THE MILDER FORMS OF PEROXISOME BIOGENESIS DISORDER^*

Chiharu Fujiwara^§, Atsushi Imamura^¶, Noriyo Hashiguchi, Nobuyuki Shimozawa^¶, Yasuyuki Suzuki^¶, Naomi Kondo^¶, Tsuneo Imanaka, Toshiro Tsukamoto, and Takashi Osumi^**

From the  Department of Life Science, Faculty of Science, Himeji Institute of Technology, 3-2-1 Koto, Kamigori, Hyogo 678-1297, the ^¶ Department of Pediatrics, Gifu University School of Medicine, 40 Tsukasa-Machi, Gifu 500-8076, and the  Department of Biological Chemistry, Faculty of Pharmaceutical Sciences, Toyama Medical and Pharmaceutical University, 2630 Sugitani, Toyama 930-0194, Japan

We established a Chinese hamster ovary cell line having a temperature-sensitive phenotype in peroxisome biogenesis. This mutant (65TS) was produced by transforming a PEX2-defective mutant, Z65, with a mutant PEX2 gene, PEX2^E55K, derived from a patient with infantile Refsum disease, a milder form of peroxisome biogenesis disorder. In 65TS, catalase was found in the cytosol at a nonpermissive temperature (39 °C), but upon the shift to a permissive temperature (33 °C), catalase gradually localized to the structures containing a 70-kDa peroxisomal membrane protein, PMP70. In contrast to catalase, other matrix proteins containing typical peroxisome targeting signals, acyl-CoA oxidase and peroxisomal 3-ketoacyl-CoA thiolase, were co-localized with PMP70 in most cells, even at 39 °C. We found that these structures are partially functional peroxisomes and named them "catalase-less peroxisomes." Catalase-less peroxisomes were also observed in human fibroblasts from patients with milder forms of peroxisome biogenesis disorder, including the one from which the mutant PEX2 gene was derived. We suggest that these structures are the causes of the milder phenotypes of the patients. Temperature-dependent restoration of the peroxisomes in 65TS occurred even in the presence of cycloheximide, a protein synthesis inhibitor. Thus, we conclude that in 65TS, catalase-less peroxisomes are the direct precursors of peroxisomes.

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