TIMM23 Gene

Name	translocase of inner mitochondrial membrane 23 homolog (yeast)
Description	The protein encoded by this gene is part of a complex located in the inner mitochondrial membrane that mediates the transport of transit peptide-containing proteins across the membrane. Multiple transcript variants, one protein-coding and others not protein-coding, have been found for this gene. [provided by RefSeq, Jul 2012]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nThe TIM23 complex is a central component of the mitochondrial protein import machinery in eukaryotes. It functions as the channel‐forming subunit that mediates the translocation of nuclear‐encoded proteins bearing an amino‐terminal presequence from the intermembrane space into the matrix or integration into the inner membrane. In this context, TIMM23 interacts with factors such as TIM21 to coordinate early assembly intermediates of the respiratory-chain complexes, thereby linking mitochondrial translation and the import of nuclear-encoded subunits, while its distinct assemblies (complexes B1, B2, and A) underscore its dual roles in maintaining housekeeping functions and, in certain nonessential forms, modulating oncogenic signaling."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its canonical import function, modulating TIM23-dependent protein sorting has emerged as a promising therapeutic strategy. For example, enhancing the sorting of nuclear-encoded mitochondrial proteins via TIM23 has been shown to ameliorate deficits related to ATP synthase deficiencies, while aberrant interactions of mutant huntingtin with the TIM23 complex compromise synaptic mitochondrial protein import and contribute to neurodegeneration. Moreover, under oxidative stress conditions, the TIM23/PAM machinery enables the rapid translocation of repair proteins such as APE1 from the intermembrane space into the matrix, thus contributing to mitochondrial genome integrity."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "3", "end_ref": "5"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nThe nuclear regulation of mitochondrial protein import is also evident at the transcriptional level; the promoter regions of the human TIM23 orthologs (TIMM23 and TIMM23B) harbor binding sites for transcription factors such as GABP and RBPJ, ensuring their expression is coupled to cellular metabolic and biogenic demands. In addition, comprehensive reviews of the human TIM23 import pathway, including insights into pathogenic mutations in components like TIM50, further emphasize its essential role in maintaining mitochondrial function and overall cellular homeostasis."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "6"}]}, {"type": "t", "text": ""}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "David U Mick, Sven Dennerlein, Heike Wiese, et al. "}, {"type": "b", "children": [{"type": "t", "text": "MITRAC links mitochondrial protein translocation to respiratory-chain assembly and translational regulation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.cell.2012.11.053"}], "href": "https://doi.org/10.1016/j.cell.2012.11.053"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23260140"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23260140"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Devanjan Sinha, Shubhi Srivastava, Lekshmi Krishna, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Unraveling the intricate organization of mammalian mitochondrial presequence translocases: existence of multiple translocases for maintenance of mitochondrial function."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cell Biol (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1128/MCB.01527-13"}], "href": "https://doi.org/10.1128/MCB.01527-13"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24636990"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24636990"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Raeka S Aiyar, Maria Bohnert, Stéphane Duvezin-Caubet, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Mitochondrial protein sorting as a therapeutic target for ATP synthase disorders."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Nat Commun (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/ncomms6585"}], "href": "https://doi.org/10.1038/ncomms6585"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25519239"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25519239"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Svitlana Yablonska, Vinitha Ganesan, Lisa M Ferrando, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Proc Natl Acad Sci U S A (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1073/pnas.1904101116"}], "href": "https://doi.org/10.1073/pnas.1904101116"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31346086"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31346086"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Arianna Barchiesi, Veronica Bazzani, Vanessa Tolotto, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Mitochondrial Oxidative Stress Induces Rapid Intermembrane Space/Matrix Translocation of Apurinic/Apyrimidinic Endonuclease 1 Protein through TIM23 Complex."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Mol Biol (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.jmb.2020.11.012"}], "href": "https://doi.org/10.1016/j.jmb.2020.11.012"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "33197464"}], "href": "https://pubmed.ncbi.nlm.nih.gov/33197464"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Jesús A Prieto-Ruiz, Rafael Alis, Sandra García-Benlloch, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Expression of the human TIMM23 and TIMM23B genes is regulated by the GABP transcription factor."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biochim Biophys Acta Gene Regul Mech (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.bbagrm.2018.01.006"}], "href": "https://doi.org/10.1016/j.bbagrm.2018.01.006"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "29413900"}], "href": "https://pubmed.ncbi.nlm.nih.gov/29413900"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Sylvie Callegari, Luis Daniel Cruz-Zaragoza, Peter Rehling "}, {"type": "b", "children": [{"type": "t", "text": "From TOM to the TIM23 complex - handing over of a precursor."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biol Chem (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1515/hsz-2020-0101"}], "href": "https://doi.org/10.1515/hsz-2020-0101"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32074073"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32074073"}]}]}]}
Synonyms	TIM23
Proteins	TIM23_HUMAN
NCBI Gene ID	100287932
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Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

TIMM23 has 5,095 functional associations with biological entities spanning 9 categories (molecular profile, organism, chemical, functional term, phrase or reference, disease, phenotype or trait, structural feature, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 107 datasets.

Click the + buttons to view associations for TIMM23 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

TIMM23 Gene

Functional Associations

Please acknowledge the Harmonizome in your publications by citing the following reference(s):

	Dataset	Summary
	Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of TIMM23 gene relative to other tissues from the Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles	tissues with high or low expression of TIMM23 gene relative to other tissues from the Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles	tissue samples with high or low expression of TIMM23 gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq	tissue samples with high or low expression of TIMM23 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq dataset.
	Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of TIMM23 gene relative to other tissues from the Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles dataset.
	BioGPS Cell Line Gene Expression Profiles	cell lines with high or low expression of TIMM23 gene relative to other cell lines from the BioGPS Cell Line Gene Expression Profiles dataset.
	BioGPS Human Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of TIMM23 gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	BioGPS Mouse Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of TIMM23 gene relative to other cell types and tissues from the BioGPS Mouse Cell Type and Tissue Gene Expression Profiles dataset.
	Carcinogenome Chemical Perturbation Carcinogenicity Signatures	small molecule perturbations changing expression of TIMM23 gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of TIMM23 gene relative to other cell lines from the CCLE Cell Line Gene CNV Profiles dataset.
	CCLE Cell Line Gene Expression Profiles	cell lines with high or low expression of TIMM23 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with TIMM23 protein from the CCLE Cell Line Proteomics dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with TIMM23 gene from the CellMarker Gene-Cell Type Associations dataset.
	ChEA Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of TIMM23 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of TIMM23 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets dataset.
	ChEA Transcription Factor Targets 2022	transcription factors binding the promoter of TIMM23 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing TIMM23 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing TIMM23 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores	cellular components containing TIMM23 protein in low- or high-throughput protein localization assays from the COMPARTMENTS Experimental Protein Localization Evidence Scores dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores	cellular components co-occuring with TIMM23 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025	cellular components co-occuring with TIMM23 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
	COSMIC Cell Line Gene CNV Profiles	cell lines with high or low copy number of TIMM23 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with TIMM23 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with TIMM23 gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with TIMM23 gene/protein from the curated CTD Gene-Disease Associations dataset.
	DeepCoverMOA Drug Mechanisms of Action	small molecule perturbations with high or low expression of TIMM23 protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with TIMM23 gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores 2025 dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with TIMM23 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores 2025	diseases co-occuring with TIMM23 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.
	DisGeNET Gene-Disease Associations	diseases associated with TIMM23 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with TIMM23 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset.
	ENCODE Histone Modification Site Profiles	histone modification site profiles with high histone modification abundance at TIMM23 gene from the ENCODE Histone Modification Site Profiles dataset.
	ENCODE Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of TIMM23 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of TIMM23 gene in ChIP-seq datasets from the ENCODE Transcription Factor Targets dataset.
	ESCAPE Omics Signatures of Genes and Proteins for Stem Cells	PubMedIDs of publications reporting gene signatures containing TIMM23 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GAD Gene-Disease Associations	diseases associated with TIMM23 gene in GWAS and other genetic association datasets from the GAD Gene-Disease Associations dataset.
	GAD High Level Gene-Disease Associations	diseases associated with TIMM23 gene in GWAS and other genetic association datasets from the GAD High Level Gene-Disease Associations dataset.
	GDSC Cell Line Gene Expression Profiles	cell lines with high or low expression of TIMM23 gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.
	GeneRIF Biological Term Annotations	biological terms co-occuring with TIMM23 gene in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.
	GeneSigDB Published Gene Signatures	PubMedIDs of publications reporting gene signatures containing TIMM23 from the GeneSigDB Published Gene Signatures dataset.