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    Pmpca peptidase (mitochondrial processing) alpha [ Mus musculus (house mouse) ]

    Gene ID: 66865, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Pmpcaprovided by MGI
    Official Full Name
    peptidase (mitochondrial processing) alphaprovided by MGI
    Primary source
    MGI:MGI:1918568
    See related
    Ensembl:ENSMUSG00000026926 AllianceGenome:MGI:1918568
    Gene type
    protein coding
    RefSeq status
    VALIDATED
    Organism
    Mus musculus
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
    Also known as
    P-55; Alpha-MPP; 1200002L24Rik; 4933435E07Rik
    Summary
    Predicted to enable endopeptidase activity. Predicted to contribute to metallopeptidase activity. Predicted to be involved in protein processing involved in protein targeting to mitochondrion. Located in mitochondrion. Is expressed in genitourinary system. Human ortholog(s) of this gene implicated in autosomal recessive spinocerebellar ataxia 2. Orthologous to human PMPCA (peptidase, mitochondrial processing subunit alpha). [provided by Alliance of Genome Resources, Dec 2024]
    Expression
    Ubiquitous expression in placenta adult (RPKM 35.0), CNS E14 (RPKM 31.1) and 28 other tissues See more
    Orthologs
    human all
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    Genomic context

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    See Pmpca in Genome Data Viewer
    Location:
    2 A3; 2 18.88 cM
    Exon count:
    17
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCm39 (GCF_000001635.27) 2 NC_000068.8 (26275554..26287134)
    108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 2 NC_000068.7 (26389348..26397121)

    Chromosome 2 - NC_000068.8Genomic Context describing neighboring genes Neighboring gene predicted gene 13562 Neighboring gene caspase recruitment domain family, member 9 Neighboring gene small nuclear RNA activating complex, polypeptide 4 Neighboring gene endosome associated trafficking regulator 1 Neighboring gene inositol polyphosphate-5-phosphatase E Neighboring gene STARR-positive B cell enhancer ABC_E5900 Neighboring gene SEC16 homolog A, endoplasmic reticulum export factor Neighboring gene RIKEN cDNA 0610009E02 gene Neighboring gene STARR-positive B cell enhancer ABC_E1241 Neighboring gene STARR-seq mESC enhancer starr_03982 Neighboring gene CapStarr-seq enhancer MGSCv37_chr2:26316530-26316713 Neighboring gene notch 1 Neighboring gene microRNA 6996

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. OxLDL triggers retrograde translocation of arginase2 in aortic endothelial cells via ROCK and mitochondrial processing peptidase. Pandey D, et al. Circ Res, 2014 Aug 1. PMID 24903103, Free PMC Article
    2. Autocatalytic processing of m-AAA protease subunits in mitochondria. Koppen M, et al. Mol Biol Cell, 2009 Oct. PMID 19656850, Free PMC Article
    3. Proteins associated with type II bone morphogenetic protein receptor (BMPR-II) and identified by two-dimensional gel electrophoresis and mass spectrometry. Hassel S, et al. Proteomics, 2004 May. PMID 15188402
    4. The ATG5 interactome links clathrin-mediated vesicular trafficking with the autophagosome assembly machinery. Baines K, et al. Autophagy Rep, 2022. PMID 35449600, Free PMC Article
    5. A mitochondrial protein compendium elucidates complex I disease biology. Pagliarini DJ, et al. Cell, 2008 Jul 11. PMID 18614015, Free PMC Article

    See all (22) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. OxLDL triggers retrograde translocation of arginase2 in aortic endothelial cells via ROCK and mitochondrial processing peptidase.
      Title: OxLDL triggers retrograde translocation of arginase2 in aortic endothelial cells via ROCK and mitochondrial processing peptidase.
    2. Data show that Afg3l1 or Afg3l2 are required for maturation of newly imported paraplegin subunits after their cleavage by MPP.
      Title: Autocatalytic processing of m-AAA protease subunits in mitochondria.
    Submit: New GeneRIF Correction

    Variation

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    Alleles

    Alleles of this type are documented at Mouse Genome Informatics  (MGI)
    • Endonuclease-mediated (1) 

    Pathways from PubChem

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    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Markers

    Homology

    Gene Ontology Provided by MGI

    Function Evidence Code Pubs
    enables endopeptidase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables metal ion binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables metalloendopeptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    contributes_to metallopeptidase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    Process Evidence Code Pubs
    involved_in protein processing ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in protein processing involved in protein targeting to mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in protein processing involved in protein targeting to mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in protein processing involved in protein targeting to mitochondrion ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    Component Evidence Code Pubs
    located_in mitochondrial inner membrane ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in mitochondrial inner membrane ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    located_in mitochondrial matrix IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in mitochondrion HDA PubMed 
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		  

    General protein information

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    Preferred Names
    mitochondrial-processing peptidase subunit alpha
    Names
    inactive zinc metalloprotease alpha
    mitochondrial matrix processing protease, alpha subunit
    NP_001405477.1
    NP_001405478.1
    NP_001405479.1
    NP_775272.1
    XP_006498300.1
    XP_036018387.1

    NCBI Reference Sequences (RefSeq)

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    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    mRNA and Protein(s)

    1. NM_001418548.1 → NP_001405477.1  mitochondrial-processing peptidase subunit alpha isoform b precursor

      Status: VALIDATED

      Source sequence(s)
      AL732541
      UniProtKB/TrEMBL
      A2AIW9, Q8BK51
      Related
      ENSMUSP00000109727.2, ENSMUST00000114093.2

    2. NM_001418549.1 → NP_001405478.1  mitochondrial-processing peptidase subunit alpha isoform c

      Status: VALIDATED

      Source sequence(s)
      AL732541

    3. NM_001418550.1 → NP_001405479.1  mitochondrial-processing peptidase subunit alpha isoform c

      Status: VALIDATED

      Source sequence(s)
      AL732541

    4. NM_173180.3 → NP_775272.1  mitochondrial-processing peptidase subunit alpha isoform a precursor

      See identical proteins and their annotated locations for NP_775272.1

      Status: VALIDATED

      Source sequence(s)
      AL732541
      Consensus CDS
      CCDS15804.1
      UniProtKB/Swiss-Prot
      Q3TF19, Q9DC61
      UniProtKB/TrEMBL
      Q3TTM6
      Related
      ENSMUSP00000075762.7, ENSMUST00000076431.13
      Conserved Domains (1) summary
      COG0612
      Location:64 → 503
      PqqL; Predicted Zn-dependent peptidase [General function prediction only]

    RNA

    1. NR_184780.1 RNA Sequence

      Status: VALIDATED

      Source sequence(s)
      AL732541

    2. NR_184781.1 RNA Sequence

      Status: VALIDATED

      Source sequence(s)
      AL732541

    RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

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

    Reference GRCm39 C57BL/6J

    Genomic

    1. NC_000068.8 Reference GRCm39 C57BL/6J

      Range
      26275554..26287134
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_036162494.1 → XP_036018387.1  mitochondrial-processing peptidase subunit alpha isoform X1

      UniProtKB/Swiss-Prot
      Q3TF19, Q9DC61
      UniProtKB/TrEMBL
      Q3TTM6
      Conserved Domains (1) summary
      COG0612
      Location:64 → 503
      PqqL; Predicted Zn-dependent peptidase [General function prediction only]

    2. XM_006498237.5 → XP_006498300.1  mitochondrial-processing peptidase subunit alpha isoform X2

      Conserved Domains (1) summary
      COG0612
      Location:1 → 355
      PqqL; Predicted Zn-dependent peptidase [General function prediction only]
    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q9DC61.1 GenPept UniProtKB/Swiss-Prot:Q9DC61

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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