ETFDH

Chr 4AR

electron transfer flavoprotein dehydrogenase

Also known as: ETFQO, MADD

The protein accepts electrons from electron transfer flavoprotein (ETF) and reduces ubiquinone in the mitochondrial respiratory chain. Mutations cause glutaric acidemia IIC, an autosomal recessive disorder of fatty acid oxidation. Loss of function mutations disrupt the electron transport chain, leading to impaired fatty acid beta-oxidation and accumulation of organic acids.

GeneReviewsOMIMResearchSummary from OMIM, UniProt
LOFmechanismARLOEUF 1.041 OMIM phenotype
Clinical SummaryETFDH
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Gene-Disease Validity (ClinGen)
multiple acyl-CoA dehydrogenase deficiency · 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.
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GeneReview available — ETFDH
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.04LOEUF
pLI 0.000
Z-score 1.36
OE 0.74 (0.541.04)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.39Z-score
OE missense 0.94 (0.861.03)
314 obs / 334.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.74 (0.541.04)
00.351.4
Missense OE0.94 (0.861.03)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 24 / 32.3Missense obs/exp: 314 / 334.2Syn Z: -0.16

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ETFDH · 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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