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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TIMM23
translocase of inner mitochondrial membrane 23
Chromosome 10 · 10q11.22
NCBI Gene: 100287932Ensembl: ENSG00000265354.5HGNC: HGNC:17312UniProt: O14925
80PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingtype 2 mitophagymitochondrionmitochondrial inner membraneneurodegenerative diseaseneoplasmnon-small cell lung carcinomaosteosarcoma
✦AI Summary

TIMM23 (translocase of inner mitochondrial membrane 23) is an essential component of the TIM23 complex, which mediates translocation of transit peptide-containing proteins across the mitochondrial inner membrane 1. Beyond protein import, TIMM23 plays a critical role in mitochondrial quality control through mitophagy regulation. TIMM23 protects PINK1 from OMA1-mediated degradation and facilitates its accumulation at the outer mitochondrial membrane in response to mitochondrial depolarization, thereby activating stress-induced mitophagy 2. This function is particularly relevant in neurodegenerative diseases; impaired mitophagy due to defective PINK1-PRKN signaling is implicated in Parkinson disease pathogenesis and Alzheimer disease 2. TIMM23 also functions within the broader mitochondrial quality control network, responding to import defects through protease-mediated regulation 3. When mitochondrial import is blocked, YME1L1 selectively degrades TIMM23 components, preventing accumulation of stalled precursors and protecting cellular viability. Dysfunction of TIMM23-dependent processes contributes to mitochondrial homeostasis failure in various pathological contexts, including drug-induced liver injury and intervertebral disc degeneration 45. Thus, TIMM23 integrates protein translocation and mitophagy functions essential for organellar quality control and disease prevention.

Sources cited
1
TIMM23 is a core component of mitochondrial protein import machinery mediating protein translocation across inner membrane
PMID: 28301740
2
TIMM23 protects PINK1 from degradation and facilitates its accumulation at outer mitochondrial membrane to activate mitophagy
PMID: 39051473
3
YME1L1 protease selectively degrades TIMM23 components when mitochondrial protein import is blocked
PMID: 39774271
4
TIMM23 is involved in mitochondrial homeostasis and mitophagy-mediated protection in drug-induced liver injury
PMID: 39099169
5
TIMM23 participates in mitochondrial quality control relevant to intervertebral disc degeneration
PMID: 37876250
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.45Moderate
neoplasmOpen Targets
0.09Suggestive
non-small cell lung carcinomaOpen Targets
0.07Suggestive
osteosarcomaOpen Targets
0.07Suggestive
cancerOpen Targets
0.05Suggestive
Alopecia universalisOpen Targets
0.05Suggestive
hypotrichosis simplexOpen Targets
0.04Suggestive
lung adenocarcinomaOpen Targets
0.04Suggestive
Trichodysplasia - xerodermaOpen Targets
0.03Suggestive
trichodysplasia-xeroderma syndromeOpen Targets
0.03Suggestive
alopecia universalis congenitaOpen Targets
0.03Suggestive
hypotrichosis 4Open Targets
0.03Suggestive
uncombable hair syndromeOpen Targets
0.03Suggestive
pure hair and nail ectodermal dysplasiaOpen Targets
0.03Suggestive
hypotrichosis 5Open Targets
0.03Suggestive
Clouston syndromeOpen Targets
0.03Suggestive
Abnormal sperm morphologyOpen Targets
0.02Suggestive
asbestosisOpen Targets
0.02Suggestive
ovarian carcinomaOpen Targets
0.02Suggestive
gliomaOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CYC1Protein interaction100%SLC25A6Protein interaction99%HSPA9Protein interaction99%TOMM70Protein interaction99%TOMM40Protein interaction99%SAMM50Protein interaction99%
Tissue Expression6 tissues
Heart
100%
Brain
78%
Bone Marrow
66%
Liver
59%
Lung
31%
Ovary
23%
Gene Interaction Network
Click a node to explore
TIMM23CYC1SLC25A6HSPA9TOMM70TOMM40SAMM50
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O14925
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.80LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.51 [0.34–0.80]
RankingsWhere TIMM23 stands among ~20K protein-coding genes
  • #5,972of 20,598
    Most Researched80
  • #6,641of 17,882
    Most Constrained (LOEUF)0.80
Genes detectedTIMM23
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The nucleoprotein of influenza A virus inhibits the innate immune response by inducing mitophagy.
PMID: 36588386
Autophagy · 2023
1.00
2
Mitochondrial Machineries for Protein Import and Assembly.
PMID: 28301740
Annu Rev Biochem · 2017
0.90
3
Spautin-1 promotes PINK1-PRKN-dependent mitophagy and improves associative learning capability in an alzheimer disease animal model.
PMID: 39051473
Autophagy · 2024
0.80
4
Organelle-specific autophagy in inflammatory diseases: a potential therapeutic target underlying the quality control of multiple organelles.
PMID: 32048886
Autophagy · 2021
0.70
5
The NLRX1-SLC39A7 complex orchestrates mitochondrial dynamics and mitophagy to rejuvenate intervertebral disc by modulating mitochondrial Zn
PMID: 37876250
Autophagy · 2024
0.60