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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TIMM13
translocase of inner mitochondrial membrane 13
Chromosome 19 · 19p13.3
NCBI Gene: 26517Ensembl: ENSG00000099800.9HGNC: HGNC:11816UniProt: Q9Y5L4
93PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmitochondrionmitochondrial inner membranemitochondrial intermembrane space chaperone complexneurodegenerative diseasediabetes mellitusatrial fibrillationlung cancer
✦AI Summary

TIMM13 is a mitochondrial intermembrane space chaperone that mediates protein import into the inner mitochondrial membrane. It functions as part of the TIMM8A-TIMM13 70 kDa complex, which facilitates import of multi-pass transmembrane proteins including TIMM23, citrin, and aralar1, while protecting hydrophobic precursors from aggregation 12. TIMM13 plays critical roles in mitochondrial biogenesis and energy metabolism through the aspartate-malate NADH shuttle 2. In cancer pathology, TIMM13 is frequently upregulated and promotes tumorigenesis through multiple mechanisms. In osteosarcoma, TIMM13 overexpression enhances ATP production and activates Akt-mTOR signaling, driving cell proliferation and migration, while TIMM13 depletion causes mitochondrial dysfunction and apoptosis 3. In lung cancer, the TIMM8A-TIMM13 complex regulates cyclin D1 and CDK6 expression, promoting cell cycle progression 4. TIMM13 expression can be dysregulated through alternative transcription initiation, increasing protein production independent of overall expression changes 5. In neurological disease, TIMM13 downregulation contributes to Alzheimer's disease pathogenesis; miR-1273g-3p suppresses TIMM13 expression, impairing mitochondrial function and promoting amyloid-beta production 6. Additionally, TIMM8A mutations in deafness-dystonia-optic neuropathy syndrome destabilize the TIMM8A-TIMM13 complex, reducing TIMM13 protein levels and causing neurological dysfunction 7.

Sources cited
1
TIMM13 assembles with TIMM8a in a 70 kDa complex in the intermembrane space and mediates import of TIMM23 and other inner membrane proteins
PMID: 11875042
2
TIMM8a-TIMM13 complex facilitates import of calcium-binding aspartate/glutamate carriers (citrin and aralar1) involved in NADH shuttling
PMID: 15254020
3
TIMM13 upregulation promotes osteosarcoma cell growth through increased ATP production and Akt-mTOR activation; TIMM13 depletion causes mitochondrial dysfunction and apoptosis
PMID: 37407582
4
TIMM8A-TIMM13 complex is elevated in lung cancer and regulates cyclin D1 and CDK6, promoting cell proliferation and migration
PMID: 40918471
5
TIMM13 5' UTR shortening via alternative transcription initiation increases protein production and promotes lung cancer cell proliferation and migration
PMID: 38985880
6
miR-1273g-3p targets TIMM13, and reduced TIMM13 expression in Alzheimer's disease promotes mitochondrial impairment and amyloid-beta production
PMID: 34685681
7
TIMM8A mutations reduce TIMM13 protein levels without affecting TIMM13 transcription, causing deafness-dystonia-optic neuropathy syndrome
PMID: 31903733
8
TIMM13 is a target of miR-34a downregulation in neuroblastoma and correlates with worse clinical outcomes
PMID: 24912852
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.36Weak
diabetes mellitusOpen Targets
0.29Weak
atrial fibrillationOpen Targets
0.09Suggestive
lung cancerOpen Targets
0.09Suggestive
osteosarcomaOpen Targets
0.08Suggestive
infectionOpen Targets
0.05Suggestive
hepatorenocardiac degenerative fibrosisOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.04Suggestive
Testicular regression syndromeOpen Targets
0.04Suggestive
Mayer-Rokitansky-Kuster-Hauser syndromeOpen Targets
0.04Suggestive
Mayer-Rokitansky-Küster-Hauser syndromeOpen Targets
0.04Suggestive
Mayer-Rokitansky-Kuster-Hauser syndrome type 1Open Targets
0.04Suggestive
polycystic kidney disease 5Open Targets
0.04Suggestive
46,XX ovotesticular disorder of sex developmentOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
testicular agenesisOpen Targets
0.03Suggestive
nephronophthisisOpen Targets
0.03Suggestive
nephronophthisis 3Open Targets
0.03Suggestive
Senior-Boichis syndromeOpen Targets
0.03Suggestive
polycystic kidney disease 8Open Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TIMM29Shared pathway100%TIMM8BProtein interaction100%TIMM10Protein interaction100%TIMM9Protein interaction100%TIMM23Protein interaction99%SLC25A12Protein interaction97%
Tissue Expression6 tissues
Liver
100%
Ovary
46%
Lung
38%
Heart
38%
Brain
38%
Bone Marrow
36%
Gene Interaction Network
Click a node to explore
TIMM13TIMM29TIMM8BTIMM10TIMM9TIMM23SLC25A12
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9Y5L4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.52LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.50–1.52]
RankingsWhere TIMM13 stands among ~20K protein-coding genes
  • #5,166of 20,598
    Most Researched93
  • #15,279of 17,882
    Most Constrained (LOEUF)1.52
Genes detectedTIMM13
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Pan-cancer transcriptome analysis reveals widespread regulation through alternative tandem transcription initiation.
PMID: 38985880
Sci Adv · 2024
1.00
2
HOXC13-driven TIMM13 overexpression promotes osteosarcoma cell growth.
PMID: 37407582
Cell Death Dis · 2023
0.90
3
Enhanced Expression of microRNA-1273g-3p Contributes to Alzheimer's Disease Pathogenesis by Regulating the Expression of Mitochondrial Genes.
PMID: 34685681
Cells · 2021
0.80
4
Functional analysis of a novel mutation in the TIMM8A gene that causes deafness-dystonia-optic neuronopathy syndrome.
PMID: 31903733
Mol Genet Genomic Med · 2020
0.70
5
Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex.
PMID: 11875042
Hum Mol Genet · 2002
0.60