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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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TIMM17A
translocase of inner mitochondrial membrane 17A
Chromosome 1 · 1q32.1
NCBI Gene: 10440Ensembl: ENSG00000134375.12HGNC: HGNC:17315UniProt: Q99595
37PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
mitochondrionmitochondrial inner membraneTIM23 mitochondrial import inner membrane translocase complextransmembrane protein transporter activityobesityneurodegenerative diseasebreast cancerlung adenocarcinoma
✦AI Summary

TIMM17A is an essential component of the TIM23 complex, a mitochondrial inner membrane translocase that mediates the import of transit peptide-containing proteins across the mitochondrial inner membrane into the matrix 1. As one of two human orthologs of yeast Tim17, TIMM17A forms highly conserved core TIM23 variants with TIMM23 and ROMO1, with structural and mechanistic features preserved across species 1. TIMM17A stability is tightly regulated by mitochondrial quality control mechanisms: unoccupied TIM23 complexes expose a C-terminal degron on TIMM17A for ATP-dependent degradation by the protease YME1L1 2, while the prohibitin complex and OCIAD1 stabilize the TIMM17A-containing translocase variant to protect it from YME1L1-mediated degradation 3. Clinically, TIMM17A dysregulation is associated with multiple pathologies. Downregulation of TIMM17A occurs in heart failure from both ischemic and dilated cardiomyopathy, contributing to mitochondrial quality control defects 4. Conversely, elevated TIMM17A expression represents an adverse prognostic marker in breast cancer and lung adenocarcinoma, associating with higher tumor grade, nodal involvement, metastasis potential, and reduced overall survival 567. MicroRNA-593-3p suppresses breast cancer growth and metastasis by targeting TIMM17A 8, suggesting therapeutic potential for modulating TIMM17A expression in cancer treatment.

Sources cited
1
TIMM17A is a core component of the TIM23 complex that mediates mitochondrial protein import; structure is highly conserved with yeast orthologs
PMID: 38837610
2
Unoccupied TIM23 complexes expose a C-terminal degron on TIMM17A for degradation by YME1L1 protease as part of mitochondrial quality control
PMID: 39774271
3
Prohibitins and OCIAD1 stabilize TIMM17A-containing TIM23 variants and protect TIMM17A from YME1L1-mediated degradation
PMID: 39630581
4
TIMM17A is downregulated in heart failure patients with ischemic and dilated cardiomyopathy, contributing to mitochondrial dysfunction
PMID: 37403271
5
High TIMM17A expression correlates with adverse pathological features (tumor grade, nodal involvement, TNM stage) and poor overall survival in breast cancer
PMID: 20972741
6
TIMM17A is upregulated in lung adenocarcinoma and associated with clinical staging, lymph node metastasis, and reduced overall and progression-free survival
PMID: 38632467
7
Elevated co-expression of TIMM17A and NMT1 is associated with worse overall survival, disease-specific survival, and disease-free survival in lung adenocarcinoma
PMID: 41083501
8
MicroRNA-593-3p suppresses breast cancer cell growth, proliferation, and invasion by downregulating TIMM17A expression
PMID: 40307502
Disease Associationsⓘ20
obesityOpen Targets
0.29Weak
neurodegenerative diseaseOpen Targets
0.24Weak
breast cancerOpen Targets
0.09Suggestive
lung adenocarcinomaOpen Targets
0.08Suggestive
Abnormality of the skeletal systemOpen Targets
0.05Suggestive
lung carcinomaOpen Targets
0.03Suggestive
atrial fibrillationOpen Targets
0.03Suggestive
Transient global amnesiaOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.02Suggestive
colorectal cancerOpen Targets
0.02Suggestive
jaw diseaseOpen Targets
0.02Suggestive
lung cancerOpen Targets
0.02Suggestive
urinary tract infectionOpen Targets
0.02Suggestive
cancerOpen Targets
0.02Suggestive
breast carcinomaOpen Targets
0.01Suggestive
colorectal carcinomaOpen Targets
0.01Suggestive
intrahepatic cholestasisOpen Targets
0.01Suggestive
breast neoplasmOpen Targets
0.01Suggestive
lymph node metastatic carcinomaOpen Targets
0.01Suggestive
hypertensionOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SLC25A6Protein interaction100%HSPA9Protein interaction100%TOMM70Protein interaction100%CYC1Protein interaction100%DNAJC19Protein interaction98%HSPA4Protein interaction98%
Tissue Expression6 tissues
Heart
100%
Liver
70%
Bone Marrow
64%
Brain
61%
Lung
37%
Ovary
25%
Gene Interaction Network
Click a node to explore
TIMM17ASLC25A6HSPA9TOMM70CYC1DNAJC19HSPA4
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q99595
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.95LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.57 [0.36–0.95]
RankingsWhere TIMM17A stands among ~20K protein-coding genes
  • #10,709of 20,598
    Most Researched37
  • #8,905of 17,882
    Most Constrained (LOEUF)0.95
Genes detectedTIMM17A
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Mitochondrial YME1L1 governs unoccupied protein translocase channels.
PMID: 39774271
Nat Cell Biol · 2025
1.00
2
OCIAD1 and prohibitins regulate the stability of the TIM23 protein translocase.
PMID: 39630581
Cell Rep · 2024
0.90
3
Heart failure in patients is associated with downregulation of mitochondrial quality control genes.
PMID: 37403271
Eur J Clin Invest · 2023
0.80
4
High TIMM17A expression is associated with adverse pathological and clinical outcomes in human breast cancer.
PMID: 20972741
Breast Cancer · 2012
0.70
5
MicroRNA-593-3p Inhibits Growth and Metastasis of Human Breast Cancer Cells by Regulating TIMM17A Expression.
PMID: 40307502
Biochem Genet · 2025
0.60