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    PCP1 rhomboid protease PCP1 [ Saccharomyces cerevisiae S288C ]

    Gene ID: 852993, updated on 9-Dec-2024

    Summary

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    Official Symbol
    PCP1
    Official Full Name
    rhomboid protease PCP1
    Primary source
    SGD:S000003333
    Locus tag
    YGR101W
    See related
    AllianceGenome:SGD:S000003333; FungiDB:YGR101W; VEuPathDB:YGR101W
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Saccharomyces cerevisiae S288C (strain: S288C)
    Lineage
    Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
    Also known as
    MDM37; RBD1; UGO2
    Summary
    Enables serine-type endopeptidase activity. Involved in regulation of mitochondrion organization and signal peptide processing. Located in mitochondrial inner membrane. Human ortholog(s) of this gene implicated in Leber hereditary optic neuropathy; coronary artery disease; hyperinsulinism; myopia; and type 2 diabetes mellitus. Orthologous to human PARL (presenilin associated rhomboid like). [provided by Alliance of Genome Resources, Dec 2024]
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    Genomic context

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    See PCP1 in Genome Data Viewer
    Location:
    chromosome: VII
    Exon count:
    1
    Sequence:
    Chromosome: VII; NC_001139.9 (693363..694403)

    Chromosome VII - NC_001139.9Genomic Context describing neighboring genes Neighboring gene Tel2p Neighboring gene GTPase-activating protein MDR1 Neighboring gene glutamyl-tRNA(Gln) amidotransferase subunit F Neighboring gene mRNA-binding ribosome synthesis protein NOP7

    Genomic regions, transcripts, and products

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    Genomic Sequence:
    NC_001139.9

    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Mitochondrial signal peptidases of yeast: the rhomboid peptidase Pcp1 and its substrate cytochrome C peroxidase. Michaelis G, et al. Gene, 2005 Jul 18. PMID 15979251
    2. Single amino acid mutations in the Saccharomyces cerevisiae rhomboid peptidase, Pcp1p, alter mitochondrial morphology. Huddleston ME, et al. Cell Biol Int, 2020 Jan. PMID 31441130, Free PMC Article
    3. Processing of Mgm1 by the rhomboid-type protease Pcp1 is required for maintenance of mitochondrial morphology and of mitochondrial DNA. Herlan M, et al. J Biol Chem, 2003 Jul 25. PMID 12707284
    4. Alternative topogenesis of Mgm1 and mitochondrial morphology depend on ATP and a functional import motor. Herlan M, et al. J Cell Biol, 2004 Apr 26. PMID 15096522, Free PMC Article
    5. Intramembrane proteolysis of Mgm1 by the mitochondrial rhomboid protease is highly promiscuous regarding the sequence of the cleaved hydrophobic segment. Schäfer A, et al. J Mol Biol, 2010 Aug 13. PMID 20558178

    See all (55) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. transmembrane segments of inner membrane proteins that are normally not processed by Pcp1 become cleavable when put in place of the authentic 'rhomboid cleavage region' (RCR) of Mgm1
      Title: Intramembrane proteolysis of Mgm1 by the mitochondrial rhomboid protease is highly promiscuous regarding the sequence of the cleaved hydrophobic segment.
    Submit: New GeneRIF Correction

    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Homology

    Gene Ontology Provided by SGD

    Function Evidence Code Pubs
    enables serine-type endopeptidase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables serine-type endopeptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables serine-type endopeptidase activity IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    enables serine-type peptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    Process Evidence Code Pubs
    involved_in proteolysis IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in regulation of mitochondrion organization IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in signal peptide processing IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in signal peptide processing IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    Component Evidence Code Pubs
    located_in membrane IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in mitochondrial inner membrane IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    located_in mitochondrial inner membrane IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in mitochondrion IEA
    Inferred from Electronic Annotation
    more info
    		  

    General protein information

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    Preferred Names
    rhomboid protease PCP1
    NP_011615.1
    • 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

    NCBI Reference Sequences (RefSeq)

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    Genome Annotation

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference assembly

    Genomic

    1. NC_001139.9 Reference assembly

      Range
      693363..694403
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001181230.1 → NP_011615.1  TPA: rhomboid protease PCP1 [Saccharomyces cerevisiae S288C]

      See identical proteins and their annotated locations for NP_011615.1

      Status: REVIEWED

      UniProtKB/Swiss-Prot
      D6VUN3, P53259
      UniProtKB/TrEMBL
      A6ZV83, B3LIC5, C7GWW2, C8Z8Z4, N1P401
      Conserved Domains (1) summary
      pfam01694
      Location:186 → 329
      Rhomboid; Rhomboid family
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    P53259.1

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