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    CLPX caseinolytic mitochondrial matrix peptidase chaperone subunit X [ Homo sapiens (human) ]

    Gene ID: 10845, updated on 4-Jan-2025

    Summary

    Official Symbol
    CLPXprovided by HGNC
    Official Full Name
    caseinolytic mitochondrial matrix peptidase chaperone subunit Xprovided by HGNC
    Primary source
    HGNC:HGNC:2088
    See related
    Ensembl:ENSG00000166855 MIM:615611; AllianceGenome:HGNC:2088
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    EPP2
    Summary
    The protein encoded by this gene is part of a protease found in mitochondria. This protease is ATP-dependent and targets specific proteins for degradation. The protease consists of two heptameric rings of the CLPP catalytic subunit sandwiched between two hexameric rings of the chaperone subunit encoded by this gene. Targeted proteins are unwound by this protein and then passed on to the CLPP subunit for degradation. Two transcript variants, one protein-coding and the other non-protein coding, have been found for this gene. [provided by RefSeq, Nov 2015]
    Expression
    Ubiquitous expression in liver (RPKM 13.6), testis (RPKM 11.0) and 25 other tissues See more
    Orthologs
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    Genomic context

    See CLPX in Genome Data Viewer
    Location:
    15q22.31
    Exon count:
    14
    Annotation release Status Assembly Chr Location
    RS_2024_08 current GRCh38.p14 (GCF_000001405.40) 15 NC_000015.10 (65148219..65185342, complement)
    RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) 15 NC_060939.1 (62957572..62994692, complement)
    RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) 15 NC_000015.9 (65440557..65477680, complement)

    Chromosome 15 - NC_000015.10Genomic Context describing neighboring genes Neighboring gene H3K4me1 hESC enhancer GRCh37_chr15:65385262-65385762 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr15:65386379-65386880 Neighboring gene ubiquitin associated protein 1 like Neighboring gene programmed cell death 7 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 6551 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 6552 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 9593 Neighboring gene NANOG-H3K27ac hESC enhancer GRCh37_chr15:65477313-65478197 Neighboring gene uncharacterized LOC124903509 Neighboring gene Sharpr-MPRA regulatory region 15221 Neighboring gene cartilage intermediate layer protein

    Genomic regions, transcripts, and products

    Expression

    • Project title: Tissue-specific circular RNA induction during human fetal development
    • Description: 35 human fetal samples from 6 tissues (3 - 7 replicates per tissue) collected between 10 and 20 weeks gestational time were sequenced using Illumina TruSeq Stranded Total RNA
    • BioProject: PRJNA270632
    • Publication: PMID 26076956
    • Analysis date: Mon Apr 2 22:54:59 2018

    Bibliography

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?

    Interactions

    General gene information

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    enables ATP binding  
    enables ATP binding  
    enables ATP hydrolysis activity  
    enables ATP hydrolysis activity PubMed 
    contributes_to ATP-dependent peptidase activity PubMed 
    enables ATP-dependent protein folding chaperone  
    enables peptidase activator activity PubMed 
    enables protein binding PubMed 
    enables protein dimerization activity  
    enables unfolded protein binding  
    enables zinc ion binding  
    Component Evidence Code Pubs
    located_in cytosol  
    part_of endopeptidase Clp complex PubMed 
    part_of endopeptidase Clp complex PubMed 
    part_of mitochondrial endopeptidase Clp complex PubMed 
    located_in mitochondrial inner membrane  
    is_active_in mitochondrial matrix  
    located_in mitochondrial matrix PubMed 
    located_in mitochondrial matrix PubMed 
    located_in mitochondrial matrix  
    located_in mitochondrial nucleoid PubMed 
    located_in mitochondrion PubMed 
    located_in mitochondrion PubMed 
    located_in nucleoplasm  

    General protein information

    Preferred Names
    ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial
    Names
    ClpX caseinolytic peptidase X homolog
    ClpX caseinolytic protease X homolog
    energy-dependent regulator of proteolysis

    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_006660.5 → NP_006651.2  ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial precursor

      See identical proteins and their annotated locations for NP_006651.2

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) represents the longer, protein-coding transcript.
      Source sequence(s)
      AC013553, AJ006267, BG717994, CA945006
      Consensus CDS
      CCDS10202.1
      UniProtKB/Swiss-Prot
      A1L428, A8K8F1, B9EGI8, O76031, Q9H4D9
      UniProtKB/TrEMBL
      Q9H072
      Related
      ENSP00000300107.3, ENST00000300107.7
      Conserved Domains (4) summary
      smart01086
      Location:509 → 603
      ClpB_D2-small; C-terminal, D2-small domain, of ClpB protein
      TIGR00382
      Location:102 → 600
      clpX; endopeptidase Clp ATP-binding regulatory subunit (clpX)
      pfam07724
      Location:286 → 502
      AAA_2; AAA domain (Cdc48 subfamily)
      cl02609
      Location:104 → 132
      Zn-ribbon; C-terminal zinc ribbon domain of RNA polymerase intrinsic transcript cleavage subunit

    RNA

    1. NR_133680.2 RNA Sequence

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) uses two alternate splice junctions compared to variant 1. This variant is represented as non-coding because the use of the 5'-most expected translational start codon renders the transcript a candidate for nonsense-mediated mRNA decay (NMD).
      Source sequence(s)
      AC013553, BC144166, BG717994, CA945006

    RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2024_08

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

    Reference GRCh38.p14 Primary Assembly

    Genomic

    1. NC_000015.10 Reference GRCh38.p14 Primary Assembly

      Range
      65148219..65185342 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_011521164.4 → XP_011519466.1  ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial isoform X1

      UniProtKB/TrEMBL
      Q9H072
      Conserved Domains (4) summary
      smart01086
      Location:495 → 589
      ClpB_D2-small; C-terminal, D2-small domain, of ClpB protein
      TIGR00382
      Location:102 → 586
      clpX; endopeptidase Clp ATP-binding regulatory subunit (clpX)
      pfam07724
      Location:272 → 488
      AAA_2; AAA domain (Cdc48 subfamily)
      cl02609
      Location:104 → 132
      Zn-ribbon; C-terminal zinc ribbon domain of RNA polymerase intrinsic transcript cleavage subunit

    RNA

    1. XR_931743.4 RNA Sequence

      Related
      ENST00000558958.1

    Alternate T2T-CHM13v2.0

    Genomic

    1. NC_060939.1 Alternate T2T-CHM13v2.0

      Range
      62957572..62994692 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_054377216.1 → XP_054233191.1  ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial isoform X1

      UniProtKB/TrEMBL
      Q9H072

    RNA

    1. XR_008488914.1 RNA Sequence

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