Structure and function of Pet100p, a molecular chaperone required for the assembly of cytochrome c oxidase in Saccharomyces cerevisiae.
The assembly of cytochrome c oxidase in the inner mitochondrial membranes of eukaryotic cells requires the protein products of a large number of nuclear genes. In yeast, some of these act globally and affect the assembly of several respiratory-chain protein complexes, whereas others act in a cytochrome c oxidase-specific fashion. Many of these yeast proteins have human counterparts, which when mutated lead to energy-related diseases. One of these proteins, Pet100p, is a novel molecular chaperone that functions to incorporate a subcomplex containing cytochrome c oxidase subunits VII, VIIa and VIII into holo-(cytochrome c oxidase). Here we report the topological disposition of Pet100p in the inner mitochondrial membrane and show that its C-terminal domain is essential for its function as a cytochrome c oxidase-specific 'assembly facilitator'.
Forsha
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Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, CO 80309-0347, USA.
Church
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Wazny
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Poyton
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D000483:Alleles; D000595:Amino Acid Sequence; D017931:DNA Primers; D004271:DNA, Fungal / Q000737:chemistry / Q000235:genetics; D003576:Electron Transport Complex IV / Q000235:genetics*; D005656:Fungal Proteins / Q000737:chemistry / Q000378:metabolism*; D015971:Gene Expression Regulation, Enzymologic; D015966:Gene Expression Regulation, Fungal; D008928:Mitochondria / Q000201:enzymology; D018832:Molecular Chaperones / Q000737:chemistry / Q000378:metabolism*; D009687:Nuclear Proteins / Q000737:chemistry / Q000378:metabolism*; D012441:Saccharomyces cerevisiae / Q000201:enzymology*; D029701:Saccharomyces cerevisiae Proteins; D017422:Sequence Analysis, DNA
D017931:DNA Primers; D004271:DNA, Fungal; D005656:Fungal Proteins; D018832:Molecular Chaperones; D009687:Nuclear Proteins; C100954:PET100 protein, S cerevisiae; D029701:Saccharomyces cerevisiae Proteins; D003576:Electron Transport Complex IV