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5 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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TMEM11
transmembrane protein 11
Chromosome 17 · 17p11.2
NCBI Gene: 8834Ensembl: ENSG00000178307.11HGNC: HGNC:16823UniProt: P17152
35PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
mitochondrial inner membranemitochondrion organizationprotein bindingmitochondrionneurodegenerative diseaseleft ventricular noncompactionAdult-onset autosomal recessive sideroblastic anemiahemoglobin E-beta-thalassemia syndrome
✦AI Summary

TMEM11 is a transmembrane protein localized to the mitochondrial inner membrane that regulates mitochondrial morphology and quality control. Primary Function: TMEM11 functions as a spatial regulator of mitochondrial dynamics by controlling crista biogenesis and overall mitochondrial network architecture 12. Mechanism: TMEM11 operates through a pathway independent of canonical DRP1/MFN fission-fusion machinery to regulate mitochondrial morphogenesis 1. At the molecular level, TMEM11 forms a complex with BNIP3 and BNIP3L and spatially restricts the formation of mitophagosomes, thereby limiting receptor-mediated mitophagy during both normoxic and hypoxic conditions 3. TMEM11 suppresses BNIP3-mediated mitophagy and apoptosis, maintaining mitochondrial integrity and function 4. Loss of TMEM11 results in elongated cristae and impaired respiratory chain activity, while overexpression reduces crista length and mitochondrial diameter 2. Disease Relevance: TMEM11 dysregulation promotes cisplatin resistance in bladder cancer by stabilizing mitochondrial function under chemotherapy stress 4. TMEM11 knockdown sensitizes tumors to cisplatin treatment, and curcumin was identified as a TMEM11 inhibitor capable of restoring chemotherapy sensitivity 4. Clinical Significance: TMEM11 represents a therapeutic target for overcoming drug resistance in cancer, and genetic perturbations in TMEM11 demonstrate its importance for proper mitochondrial dynamics relevant to neurodegenerative disease pathogenesis 5.

Sources cited
1
TMEM11 promotes cisplatin resistance by inhibiting BNIP3-mediated mitophagy in bladder cancer; TMEM11 knockdown sensitizes tumors and curcumin inhibits TMEM11
PMID: 41570932
2
TMEM11 is an outer mitochondrial membrane protein that forms a complex with BNIP3/BNIP3L and spatially restricts mitophagosome formation
PMID: 36795401
3
TMEM11 (human orthologue of Drosophila PMI) regulates mitochondrial morphogenesis through a pathway independent of DRP1/MFN
PMID: 21274005
4
PMI/TMEM11 controls crista biogenesis and mitochondrial diameter; loss increases crista length while overexpression reduces it; controls respiratory chain activity
PMID: 23264743
5
TMEM11 is identified as crucial for normal mitochondrial dynamics using pooled imaging analysis of mitochondrial-related genes
PMID: 40751083
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.52Moderate
left ventricular noncompactionOpen Targets
0.07Suggestive
Adult-onset autosomal recessive sideroblastic anemiaOpen Targets
0.05Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.05Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.05Suggestive
IRIDA syndromeOpen Targets
0.05Suggestive
left ventricular noncompaction 10Open Targets
0.05Suggestive
hypertrophic cardiomyopathyOpen Targets
0.05Suggestive
dilated cardiomyopathy 1GGOpen Targets
0.04Suggestive
hemoglobin D diseaseOpen Targets
0.04Suggestive
Rh deficiency syndromeOpen Targets
0.04Suggestive
dilated cardiomyopathy 1AAOpen Targets
0.04Suggestive
Hemolytic anemia due to red cell pyruvate kinase deficiencyOpen Targets
0.04Suggestive
hemolytic anemia due to erythrocyte adenosine deaminase overproductionOpen Targets
0.04Suggestive
sick sinus syndrome 2, autosomal dominantOpen Targets
0.04Suggestive
long QT syndrome 15Open Targets
0.04Suggestive
Romano-Ward syndromeOpen Targets
0.04Suggestive
overhydrated hereditary stomatocytosisOpen Targets
0.04Suggestive
congenital heart blockOpen Targets
0.03Suggestive
atrial standstillOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RRAGAProtein interaction100%CHCHD3Protein interaction98%BNIP3Protein interaction78%HSPA9Protein interaction78%MTX2Protein interaction76%IMMTProtein interaction74%
Tissue Expression6 tissues
Lung
100%
Heart
94%
Liver
81%
Bone Marrow
73%
Brain
60%
Ovary
49%
Gene Interaction Network
Click a node to explore
TMEM11RRAGACHCHD3BNIP3HSPA9MTX2IMMT
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt P17152
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.27Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.06 [0.02–0.27]
RankingsWhere TMEM11 stands among ~20K protein-coding genes
  • #11,083of 20,598
    Most Researched35
  • #903of 17,882
    Most Constrained (LOEUF)0.27 · top 10%
Genes detectedTMEM11
Sources retrieved5 papers
Response time—
📄 Sources
5
1
TMEM11 promotes cisplatin resistance by inhibiting BNIP3-mediated mitophagy in bladder cancer.
PMID: 41570932
Cancer Lett · 2026
1.00
2
The outer mitochondrial membrane protein TMEM11 demarcates spatially restricted BNIP3/BNIP3L-mediated mitophagy.
PMID: 36795401
J Cell Biol · 2023
0.80
3
Inner-membrane proteins PMI/TMEM11 regulate mitochondrial morphogenesis independently of the DRP1/MFN fission/fusion pathways.
PMID: 21274005
EMBO Rep · 2011
0.60
4
The Drosophila inner-membrane protein PMI controls crista biogenesis and mitochondrial diameter.
PMID: 23264743
J Cell Sci · 2013
0.40
5
Pathogenic morphological signatures of perturbations in mitochondrial-related genes revealed by pooled imaging assay.
PMID: 40751083
Npj Imaging · 2025
0.20