TMEM70

Chr 8AR

transmembrane protein 70

The protein is a mitochondrial inner membrane protein involved in the biogenesis of mitochondrial ATP synthase (complex V). Mutations cause autosomal recessive mitochondrial complex V deficiency, typically presenting as neonatal mitochondrial encephalocardiomyopathy due to ATP synthase deficiency.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.921 OMIM phenotype
Clinical SummaryTMEM70
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Gene-Disease Validity (ClinGen)
mitochondrial disease · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.03) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

ncbi: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.92LOEUF
pLI 0.029
Z-score 1.75
OE 0.40 (0.200.92)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.13Z-score
OE missense 0.97 (0.841.12)
139 obs / 143.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.40 (0.200.92)
00.351.4
Missense OE0.97 (0.841.12)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 4 / 9.9Missense obs/exp: 139 / 143.4Syn Z: -0.55
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTMEM70-related mitochondrial complex V (ATP synthase) deficiency, nuclearLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.74top 25%
GOF
0.5072th %ile
LOF
0.3067th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TMEM70 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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