TSFM

Chr 12AR

Ts translation elongation factor, mitochondrial

Also known as: EFTS, EFTSMT

The protein catalyzes guanine nucleotide exchange on elongation factor Tu during mitochondrial protein translation. Mutations cause combined oxidative phosphorylation deficiency 3, an autosomal recessive disorder resulting from impaired mitochondrial protein synthesis. The pathogenic mechanism involves defective mitochondrial translation elongation leading to reduced oxidative phosphorylation complex function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 1.451 OMIM phenotype
Clinical SummaryTSFM
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Gene-Disease Validity (ClinGen)
mitochondrial disease · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.45LOEUF
pLI 0.000
Z-score 0.25
OE 0.93 (0.611.45)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.22Z-score
OE missense 1.05 (0.931.19)
177 obs / 169.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.93 (0.611.45)
00.351.4
Missense OE1.05 (0.931.19)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 14 / 15.0Missense obs/exp: 177 / 169.1Syn Z: -0.52

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TSFM · 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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