GFM1

Chr 3AR

G elongation factor mitochondrial 1

Also known as: COXPD1, EFG, EFG1, EFGM, EGF1, GFM, hEFG1, mtEF-G1

The protein functions as a mitochondrial translation elongation factor essential for protein synthesis within mitochondria. Mutations cause combined oxidative phosphorylation deficiency 1, an autosomal recessive disorder resulting from impaired mitochondrial respiratory chain function. The pathogenic mechanism involves dominant-negative effects that disrupt normal mitochondrial protein translation.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.801 OMIM phenotype
Clinical SummaryGFM1
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Gene-Disease Validity (ClinGen)
Leigh syndrome · ARModerate

Moderate evidence — consider for supplementary testing

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
0.80LOEUF
pLI 0.000
Z-score 2.65
OE 0.58 (0.430.80)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.28Z-score
OE missense 0.82 (0.750.90)
339 obs / 412.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.430.80)
00.351.4
Missense OE0.82 (0.750.90)
00.61.4
Synonymous OE1.07
01.21.6
LoF obs/exp: 27 / 46.5Missense obs/exp: 339 / 412.1Syn Z: -0.61
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveGFM1-related combined oxidative phosphorylation deficiencyLOFAR

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

DN
0.74top 25%
GOF
0.7127th %ile
LOF
0.2969th %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

GFM1 · 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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