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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
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
    Physical map
    LOC118706 10q11.22 similar to ARF GTPase-activating protein LOC387674 10 similar to Ribosome biogenesis protein BMS1 homolog MGC35285 10q11.22 hypothetical protein MGC35285 MAPK8 10q11.2 mitogen-activated protein kinase 8 RhoGAP2 LOC387675 10 LOC387675 LOC159491 LOC389964 10 similar to ribosomal protein L13a; 60S ribosomal protein L13a; 23 kD highly basic protein KIAA1607 FLJ31737 LOC170370 10q11.23 hypothetical LOC170370 LOC170371 10q11.23 hypothetical protein LOC170371 LOC118461 DRG11 ERCC6 10q11 excision repair cross-complementing rodent repair deficiency, complementation group 6 PGBD3 10q11 piggyBac transposable element derived 3 CHAT 10q11.2 choline acetyltransferase SLC18A3 10q11.2 solute carrier family 18 (vesicular acetylcholine), member 3 LOC282966 10q11.23 hypothetical protein LOC282966 FLJ10851 10q11.23 hypothetical protein FLJ10851 MAPK6PS6 10q11.21 hypothetical protein FLJ10851 PARG 10q11.23 poly (ADP-ribose) glycohydrolase LOC387676 10 similar to KIAA0592 protein LOC387677 10 hypothetical gene supported by BC051717; NM_133446 LOC255319 10q11.23 similar to ARF GTPase-activating protein MSMB 10q11.2 microseminoprotein, beta- NCOA4 10q11.2 nuclear receptor coactivator 4 TIMM23 10q11.21-q11.23 translocase of inner mitochondrial membrane 23 homolog (yeast) LOC387678 10 similar to ARF GTPase-activating protein LOC389965 10 similar to Sodium/hydrogen exchanger 3 (Na(+)/H(+) exchanger 3) (NHE-3) LOC387679 10 similar to KIAA0592 protein LOC387680 10 similar to KIAA0592 protein LOC389966 10 similar to Sodium/hydrogen exchanger 3 (Na(+)/H(+) exchanger 3) (NHE-3) ASAH2 10q11.21 N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 2 LOC387681 10 hypothetical gene supported by BT007130; NM_001378 MOB 10q11.2 mob protein LOC340858 10q21.1 similar to hypothetical protein FLJ10539 FLJ31958 10q21.1 hypothetical protein FLJ31958 LOC389967 10 similar to bA182L21.1 (novel protein similar to hypothetical proteins) LOC389968 10 similar to Cathepsin L preproprotein LOC283023 10q21.1 similar to Geranylgeranyl transferase type I beta subunit (Type I protein geranyl-geranyltransferase beta subunit) (GGTase-I-beta) ACF 10q21,1 similar to Geranylgeranyl transferase type I beta subunit (Type I protein geranyl-geranyltransferase beta subunit) (GGTase-I-beta) PRKG1 10q11.2 protein kinase, cGMP-dependent, type I CSTF2T 10q22-q23 protein kinase, cGMP-dependent, type I
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    7 - 1182 21.8 209 - -
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   highly
    Digestiveliver   lowly
    Endocrineadrenal gland   moderately
     pancreas   lowly
    Hearing/Equilibriumear   moderately
    Nervousbrain   moderately
    Reproductivefemale systemuteruscervix highly
     female systembreastmammary gland moderately
     male systemprostate   
     respiratory tractlarynx  predominantly
    Urinarykidney   lowly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / hematopoieticbone marrow   
    Connectiveadipose  highly
    cell lineage
    cell lines
    fluid/secretion blood
    at STAGE
    physiological period fetal, pregnancy
    Text lowly in placenta, highly in umbilical cord
    PHYSICAL PROPERTIES Hydrophilic Hydrophobic
  • four hydrophobic membrane-spanning segments
  • a 74-amino acid N-terminal hydrophilic segment
  • mono polymer complex
    interspecies homolog to rattus Timm23 (95.7 pc)
    homolog to murine Timm23 (97.1 pc)
  • TIM17/TIM22/TIM23 family
  • CATEGORY enzyme , transport channel
    SUBCELLULAR LOCALIZATION     intracellular
    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
    PHYSIOLOGICAL PROCESS mitochondrial transport
  • protein transport
    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)
    small molecule
  • 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
    corresponding disease(s)
    Variant & Polymorphism
    Candidate gene
    Therapy target
    targeting TIMM23-dependent protein sorting improves an array of phenotypes associated with ATP synthase disorders