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Peroxisome assembly factor-2, a putative ATPase cloned by functional complementation on a peroxisome-deficient mammalian cell mutant.

Authors: Tsukamoto, T  Miura, S  Nakai, T  Yokota, S  Shimozawa, N  Suzuki, Y  Orii, T  Fujiki, Y  Sakai, F  Bogaki, A  Yasumo, H  Osumi, T 
Citation: Tsukamoto T, etal., Nat Genet 1995 Dec;11(4):395-401.
Pubmed: (View Article at PubMed) PMID:7493019
DOI: Full-text: DOI:10.1038/ng1295-395

Rat peroxisome assembly factor-2 (PAF-2) cDNA was isolated by functional complementation of peroxisome deficiency of a mutant CHO cell line, ZP92, using transient transfection assay. This cDNA encodes a 978-amino acid protein with two putative ATP-binding sites. PAF-2 is a member of a putative ATPase family, including two yeast gene products essential for peroxisome assembly. A stable transformant of ZP92 with the cDNA was morphologically and biochemically restored for peroxisome biogenesis. Fibroblasts derived from patients deficient in peroxisome biogenesis (complementation group C) were also complemented with PAF-2 cDNA, indicating that PAF-2 is a strong candidate for the pathogenic gene of group C peroxisome deficiency.

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CRRD Object Information
CRRD ID: 729462
Created: 2003-11-26
Species: All species
Last Modified: 2003-11-26
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.