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Systematic name YGR101W
Gene name PCP1
Aliases MDM37, RBD1
Feature type ORF, Verified
Coordinates Chr VII:693363..694403
Primary SGDID S000003333

Description of YGR101W: Mitochondrial serine protease required for the processing of various mitochondrial proteins and maintenance of mitochondrial DNA and morphology; belongs to the rhomboid-GlpG superfamily of intramembrane peptidases[1][2][3][4]


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Protein Details

Protein Function/Process

Limited proteolysis of Mgm1p generating the short isoform of Mgm1p depends on the mitochondrial rhomboid protease Pcp1p. Lack of Pcp1p can partially be complemented by expression of an artificial variant of the short isoform of Mgm1p. [2] [5]



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  1. Esser K, et al. (2002) A novel two-step mechanism for removal of a mitochondrial signal sequence involves the mAAA complex and the putative rhomboid protease Pcp1. J Mol Biol 323(5):835-43 SGD PMID 12417197
  2. 2.0 2.1 Herlan M, et al. (2003) Processing of Mgm1 by the rhomboid-type protease Pcp1 is required for maintenance of mitochondrial morphology and of mitochondrial DNA. J Biol Chem 278(30):27781-8 SGD PMID 12707284
  3. McQuibban GA, et al. (2003) Mitochondrial membrane remodelling regulated by a conserved rhomboid protease. Nature 423(6939):537-41 SGD PMID 12774122
  4. Sesaki H, et al. (2003) Cells lacking Pcp1p/Ugo2p, a rhomboid-like protease required for Mgm1p processing, lose mtDNA and mitochondrial structure in a Dnm1p-dependent manner, but remain competent for mitochondrial fusion. Biochem Biophys Res Commun 308(2):276-83 SGD PMID 12901865
  5. submitted by Andreas Reichert on 2003-09-25

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