Abstract
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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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ATPases Associated with Diverse Cellular Activities
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Acyl-CoA Oxidase
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Acyltransferases / metabolism
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Adenosine Triphosphatases / analysis
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Adenosine Triphosphatases / chemistry
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Adenosine Triphosphatases / genetics*
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Amino Acid Sequence
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Animals
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Base Sequence
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Binding Sites
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CHO Cells
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Catalase / analysis
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Cloning, Molecular / methods
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Cricetinae
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Cytosol / enzymology
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DNA, Complementary / genetics
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Fibroblasts
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Genetic Complementation Test*
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Humans
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Liver / chemistry
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Microbodies / enzymology*
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Molecular Sequence Data
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Mutation
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Oxidoreductases / analysis
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Peroxisomal Disorders / genetics
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Peroxisomal Disorders / metabolism
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RNA, Messenger / analysis
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Rats
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Sequence Analysis, DNA
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Sequence Homology, Amino Acid
Substances
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DNA, Complementary
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RNA, Messenger
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Oxidoreductases
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Catalase
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Acyl-CoA Oxidase
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Acyltransferases
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glycerone-phosphate O-acyltransferase
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Adenosine Triphosphatases
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ATPases Associated with Diverse Cellular Activities
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PEX6 protein, human
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Pex6 protein, rat