Peroxisome assembly factor-2, a putative ATPase cloned by functional complementation on a peroxisome-deficient mammalian cell mutant.
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.
D000074183:ATPases Associated with Diverse Cellular Activities; D042962:Acyl-CoA Oxidase; D000217:Acyltransferases / Q000378:metabolism; D000251:Adenosine Triphosphatases / Q000032:analysis / Q000737:chemistry / Q000235:genetics*; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D001665:Binding Sites; D016466:CHO Cells; D002374:Catalase / Q000032:analysis; D003001:Cloning, Molecular / Q000379:methods; D006224:Cricetinae; D003600:Cytosol / Q000201:enzymology; D018076:DNA, Complementary / Q000235:genetics; D005347:Fibroblasts; D005816:Genetic Complementation Test; D006801:Humans; D008099:Liver / Q000737:chemistry; D008830:Microbodies / Q000201:enzymology*; D008969:Molecular Sequence Data; D009154:Mutation; D010088:Oxidoreductases / Q000032:analysis; D018901:Peroxisomal Disorders / Q000235:genetics / Q000378:metabolism; D012333:RNA, Messenger / Q000032:analysis; D051381:Rats; D017422:Sequence Analysis, DNA; D017386:Sequence Homology, Amino Acid
D018076:DNA, Complementary; D012333:RNA, Messenger; D010088:Oxidoreductases; D002374:Catalase; D042962:Acyl-CoA Oxidase; D000217:Acyltransferases; C020051:glycerone-phosphate O-acyltransferase; D000251:Adenosine Triphosphatases; D000074183:ATPases Associated with Diverse Cellular Activities; C490759:PEX6 protein, human; C096998:Pex6 protein, rat