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5 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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TMEM223
transmembrane protein 223
Chromosome 11 · 11q12.3
NCBI Gene: 79064Ensembl: ENSG00000168569.8HGNC: HGNC:28464UniProt: A0PJW6
21PubMed Papers
16Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
mitochondrial ribosome bindingmitochondrial respiratory chain complex IV assemblymitochondrionmitochondrial inner membraneCachexiabreast cancerneoplasmschizophrenia
✦AI Summary

TMEM223 is a mitochondrial ribosome-associated protein that functions as a critical assembly factor for cytochrome c oxidase complex (complex IV) biogenesis 1. It stimulates translation of MT-CO1 mRNA and serves as a constituent of early COX1 assembly intermediates, linking early assembly stages to the mitochondrial translation machinery 1. TMEM223 belongs to the TMEM70/TMEM186/TMEM223 protein family, which is evolutionarily conserved exclusively in species possessing OXPHOS complexes, suggesting fundamental importance for oxidative phosphorylation 2. Beyond its mitochondrial role, TMEM223 functions as a voltage-gated Ca²⁺ channel interaction partner, lowering average current density and modulating calcium channel inactivation kinetics, potentially negatively regulating Ca²⁺ entry required for neurotransmitter release 3. Clinical relevance includes associations with cancer prognosis; lower TMEM223 expression correlates with better progression-free survival and macrophage infiltration patterns in breast cancer 4. Additionally, TMEM223 has been identified as part of a genetically regulated protein module associated with cognitive resilience in Alzheimer's disease mouse models, suggesting potential involvement in neuroprotection 5. These diverse functions reflect TMEM223's roles in both mitochondrial bioenergetics and neuronal calcium signaling.

Sources cited
1
TMEM223 is a ribosome-associated protein involved in complex IV biogenesis that stimulates COX1 mRNA translation and constitutes early COX1 assembly intermediates
PMID: 34969438
2
TMEM223 belongs to an evolutionarily conserved TMEM70/TMEM186/TMEM223 protein family found only in species with OXPHOS complexes
PMID: 32275929
3
TMEM223 interacts with voltage-gated Ca²⁺ channels (CaV2.2), lowering current density and modulating inactivation kinetics
PMID: 30612149
4
TMEM223 expression is negatively associated with macrophage proportions and positively associated with better progression-free survival in breast cancer
PMID: 32783527
5
TMEM223 is part of a genetically regulated protein module associated with cognitive resilience in Alzheimer's disease
PMID: 41225772
Disease Associationsⓘ16
CachexiaOpen Targets
0.04Suggestive
breast cancerOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.00Suggestive
schizophreniaOpen Targets
0.00Suggestive
cervical cancerOpen Targets
0.00Suggestive
esophageal cancerOpen Targets
0.00Suggestive
gastric cancerOpen Targets
0.00Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
Hereditary breast cancerOpen Targets
0.00Suggestive
hereditary breast carcinomaOpen Targets
0.00Suggestive
lung cancerOpen Targets
0.00Suggestive
melanomaOpen Targets
0.00Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.00Suggestive
ovarian serous cystadenocarcinomaOpen Targets
0.00Suggestive
thyroid cancer, nonmedullary, 1Open Targets
0.00Suggestive
uterine corpus endometrial carcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
COX17Shared pathway100%COX16Shared pathway100%COA4Shared pathway100%PET117Shared pathway100%PET100Shared pathway100%COA5Shared pathway100%
Tissue Expression6 tissues
Liver
100%
Heart
86%
Lung
61%
Ovary
57%
Brain
48%
Bone Marrow
22%
Gene Interaction Network
Click a node to explore
TMEM223COX17COX16COA4PET117PET100COA5
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt A0PJW6
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.78LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.19 [0.78–1.78]
RankingsWhere TMEM223 stands among ~20K protein-coding genes
  • #14,045of 20,598
    Most Researched21
  • #16,477of 17,882
    Most Constrained (LOEUF)1.78
Genes detectedTMEM223
Sources retrieved5 papers
Response time—
📄 Sources
5
1
Defining the interactome of the human mitochondrial ribosome identifies SMIM4 and TMEM223 as respiratory chain assembly factors.
PMID: 34969438
Elife · 2021
1.00
2
Paclitaxel Promotes Tumor-Infiltrating Macrophages in Breast Cancer.
PMID: 32783527
Technol Cancer Res Treat · 2020
0.80
3
TMEM70 functions in the assembly of complexes I and V.
PMID: 32275929
Biochim Biophys Acta Bioenerg · 2020
0.60
4
Genetic variation in the Nr1d1 transcription factor binding site shapes metabolism-related protein networks associated with cognitive resilience in an Alzheimer's disease mouse reference panel.
PMID: 41225772
Alzheimers Dement · 2025
0.40
5
Four novel interaction partners demonstrate diverse modulatory effects on voltage-gated Ca
PMID: 30612149
Pflugers Arch · 2019
0.20