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FLASH GENE
Symbol TIMM23 contributors: nptmct - updated : 22-05-2019
HGNC name translocase of inner mitochondrial membrane 23 homolog (yeast)
HGNC id 17312
Location 10q11.22      Physical location : 51.592.089 - 51.623.336
Synonym name mitochondrial import inner membrane translocase subunit Tim23
Synonym symbol(s) TIM23, PRO1197, MGC22767, FLJ40725, MGC87383, FLJ79448, FLJ57459, FLJ56773, MGC71995
DNA
TYPE functioning gene
STRUCTURE 31.48 kb     7 Exon(s)
10 Kb 5' upstream gene genomic sequence study
regulatory sequence Promoter
text structure
  • sharing a 470-bp common promoter region with PARG,
  • activity of the promoter was found to be 3.7 fold higher for TIMM23 than for PARG, indicating that the two genes are expressed at different levels
  • MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    7 - 1182 21.8 209 - -
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   highly
    Digestiveliver   lowly
    Endocrineadrenal gland   moderately
     pancreas   lowly
    Hearing/Equilibriumear   moderately
    Lymphoid/Immunespleen    
    Nervousbrain   moderately
    Reproductivefemale systemuteruscervix highly
     female systembreastmammary gland moderately
     male systemprostate   
    Respiratorylung    
     respiratory tractlarynx  predominantly
    Urinarykidney   lowly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / hematopoieticbone marrow   
    Connectiveadipose  highly
    Connectivebone   
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion blood
    at STAGE
    physiological period fetal, pregnancy
    Text lowly in placenta, highly in umbilical cord
    PROTEIN
    PHYSICAL PROPERTIES Hydrophilic Hydrophobic
    STRUCTURE
    motifs/domains
  • four hydrophobic membrane-spanning segments
  • a 74-amino acid N-terminal hydrophilic segment
  • mono polymer complex
    HOMOLOGY
    interspecies homolog to rattus Timm23 (95.7 pc)
    homolog to murine Timm23 (97.1 pc)
    Homologene
    FAMILY
  • TIM17/TIM22/TIM23 family
  • CATEGORY enzyme , transport channel
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    text multi-pass membrane protein
    basic FUNCTION
  • translocating nuclear-encoded proteins into the mitochondrion
  • mediates translocation of preproteins across and their insertion into the mitochondrial inner membrane (Popov-Celeketi&
  • 263; 2008)
  • key component of the mitochondrial import machinery (Ahting 2009)
  • TIMM23 complex translocates and inserts proteins into the mitochondrial inner membrane (Tamuar 2009)
  • seems to require the TIMM8A-TIMM13 complex for translocation across the outer membrane (Hasson 2010)
  • molecular link between the TIMM23 transport machinery and assembly of respiratory-chain complexes that regulate mitochondrial protein synthesis in response to their assembly state (PMID
  • central role of the intermembrane space domain of TIMM23 in the formation and regulation of the presequence translocase
  • majority of inner membrane protein precursors are synthesized in the cytosol, but precursors with a cleavable presequence are imported by the presequence translocase (TIMM23 complex), while other precursors containing internal targeting signals are imported by the carrier translocase (TIMM22 complex)
  • TIMM22, TIMM23 import pathways depend on the presence of a membrane potential, which is generated by the activity of the respiratory chain
  • is a key component of the mitochondrial import machinery, mediating preprotein translocation across the mitochondrial inner membrane
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS mitochondrial transport
    text
  • protein transport
  • PATHWAY
    metabolism
    signaling
    a component
  • organized into two distinct 110 kDa complexes (one containing TIMM17A and the other containing TIMM17B)
  • forming part of the TIMM23 receptor complex consisting of at least three different proteins TIMM17, TIMM23 and TIMM44
  • mediating the ATP-dependent import of proteins into mitochondria
  • MAGMAS:DNAJC19 subcomplex is associated with TIMM23 complex and thus regulates mitochondrial import process (Sinha 2010)
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • TIMM17A
  • TIMM17B
  • TIMM23-TIMM50 interactions facilitate a late step of protein translocation across the inner membrane by promoting motor functions of mitochondrial Hsp70 in the matrix (Tamura 2009)
  • TIMM44
  • TIMM21 could shuttle between TIMM23 and MITRAC complexes to fulfill a more direct function in the assembly process of nuclear-encoded subunits into assembly intermediates
  • C-terminal sequence of TIMM23 is dispensable for growth and functions as an inhibitor for binding of TIMM21
  • essential role of GABPA in activating TIMM23 expression
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    metabolism  
    targeting TIMM23-dependent protein sorting improves an array of phenotypes associated with ATP synthase disorders
    ANIMAL & CELL MODELS