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

Gene ID: 66865, updated on 3-Apr-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 female reproductive system; male reproductive system; ovary; primary sex cord; and testis. 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, Apr 2022]
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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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

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

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 (21) 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 proteolysis IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
located_in membrane IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrial inner membrane ISO
Inferred from Sequence Orthology
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
    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

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