GFER

Chr 16AR

growth factor, augmenter of liver regeneration

Also known as: ALR, ERV1, HERV1, HPO, HPO1, HPO2, HSS, MMCHD

GFER encodes a FAD-dependent sulfhydryl oxidase that regenerates disulfide bonds in the mitochondrial chaperone CHCHD4/MIA40, which is essential for protein folding in the mitochondrial intermembrane space. Autosomal recessive mutations cause mitochondrial progressive myopathy with congenital cataract and developmental delay, affecting muscle, eye, and neurodevelopmental systems from early life.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.651 OMIM phenotype
Clinical SummaryGFER
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Gene-Disease Validity (ClinGen)
mitochondrial disease · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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
1.65LOEUF
pLI 0.000
Z-score 0.30
OE 0.87 (0.481.65)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.20Z-score
OE missense 1.05 (0.901.23)
114 obs / 108.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.87 (0.481.65)
00.351.4
Missense OE1.05 (0.901.23)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 6 / 6.9Missense obs/exp: 114 / 108.3Syn Z: -0.33
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
moderateGFER-related mitochondrial progressive myopathy with congenital cataract, hearing loss and developmental delayLOFAR

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

DN
0.6743th %ile
GOF
0.5857th %ile
LOF
0.3261th %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

GFER · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC