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9 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TIMM29
translocase of inner mitochondrial membrane 29
Chromosome 19 · 19p13.2
NCBI Gene: 90580Ensembl: ENSG00000142444.7HGNC: HGNC:25152UniProt: Q9BSF4
44PubMed Papers
14Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingprotein-macromolecule adaptor activityprotein insertion into mitochondrial inner membranemitochondrionceliac diseasemucositisstomatitispsoriasis
✦AI Summary

TIMM29 (translocase of inner mitochondrial membrane 29) is a metazoan-specific subunit of the TIM22 complex, a multisubunit translocase embedded in the mitochondrial inner membrane 1. The primary function of TIMM29 is to facilitate the import and insertion of multi-pass transmembrane carrier proteins into the mitochondrial inner membrane, using the membrane potential as a driving force 1. TIMM29 is essential for TIM22 complex stability and assembly, and mediates physical contact between the TIM22 and TOM (outer membrane) complexes to enable transport of hydrophobic substrates across the intermembrane space 1. Functionally, TIMM29 supports iron-sulfur cluster biogenesis by facilitating mitochondrial iron uptake through its role in stabilizing iron transporter proteins on mitochondria, thereby promoting cell proliferation 2. Disease relevance is substantial: biallelic TIMM29 variants cause Sengers syndrome, characterized by congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, and lactic acidosis, accompanied by combined respiratory chain deficiency and abnormal mitochondrial accumulation 3. Clinically, TIMM29 also modulates hepatitis B virus replication through interactions with the HBV preS1 protein; its expression suppresses HBV amplification by upregulating antiviral factors like SRSF1 4. These findings establish TIMM29 as critical for both mitochondrial biogenesis and innate antiviral defense.

Sources cited
1
TIMM29 is a metazoan-specific TIM22 subunit required for complex stability, assembly, and TOM complex contact
PMID: 27554484
2
TIMM29 supports Fe-S biogenesis by facilitating mitochondrial iron uptake and cell proliferation
PMID: 41418755
3
Biallelic TIMM29 variants cause Sengers syndrome with cataracts, cardiomyopathy, myopathy, and respiratory chain deficiency
PMID: 40022150
4
TIMM29 overexpression inhibits HBV replication by upregulating SRSF1 expression
PMID: 38294104
5
TIMM29 interacts with HBV preS1 protein and modulates HBV life cycle
PMID: 32970362
6
TIMM29 is part of the human TIM22 complex architecture determined by crosslinking mass spectrometry
PMID: 33125709
Disease Associationsⓘ14
celiac diseaseOpen Targets
0.04Suggestive
mucositisOpen Targets
0.02Suggestive
stomatitisOpen Targets
0.02Suggestive
coronary artery diseaseOpen Targets
0.02Suggestive
psoriasisOpen Targets
0.02Suggestive
Abnormality of the gastrointestinal tractOpen Targets
0.02Suggestive
HypercholesterolemiaOpen Targets
0.02Suggestive
Pick diseaseOpen Targets
0.01Suggestive
Sengers syndromeOpen Targets
0.01Suggestive
neuroblastomaOpen Targets
0.01Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
hepatitis B virus infectionOpen Targets
0.00Suggestive
hyperinsulinemic hypoglycemia, familial, 4Open Targets
0.00Suggestive
rhabdomyolysisOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TIMM13Shared pathway100%TIMM8BShared pathway100%TIMM10Protein interaction100%TIMM9Protein interaction100%TIMM10BProtein interaction81%TIMM22Protein interaction81%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
100%
Brain
88%
Heart
79%
Lung
78%
Ovary
67%
Gene Interaction Network
Click a node to explore
TIMM29TIMM13TIMM8BTIMM10TIMM9TIMM10BTIMM22
PROTEIN STRUCTURE
Preparing viewer…
PDB7CGP · 3.70 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.92LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.52 [1.09–1.92]
RankingsWhere TIMM29 stands among ~20K protein-coding genes
  • #9,666of 20,598
    Most Researched44
  • #17,393of 17,882
    Most Constrained (LOEUF)1.92
Genes detectedTIMM29
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
The TIM22 carrier translocase supports cell proliferation by facilitating mitochondrial iron uptake for Fe-S biogenesis.
PMID: 41418755
Mol Cell · 2025
1.00
2
Sengers syndrome caused by biallelic TIMM29 variants and RNAi silencing in Drosophila orthologue recapitulates the human phenotype.
PMID: 40022150
Hum Genomics · 2025
0.89
3
TIMM29 interacts with hepatitis B virus preS1 to modulate the HBV life cycle.
PMID: 32970362
Microbiol Immunol · 2020
0.78
4
Analysis of the Functional Role of TIMM29 in the Hepatitis B Virus Life Cycle.
PMID: 39956808
Microbiol Immunol · 2025
0.67
5
Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria.
PMID: 28712724
Mol Cell · 2017
0.56