ETFB

Chr 19AR

electron transfer flavoprotein subunit beta

Also known as: FP585, MADD

The electron transfer flavoprotein beta subunit accepts electrons from multiple mitochondrial dehydrogenases involved in fatty acid oxidation and amino acid metabolism, then transfers them to the main respiratory chain. Mutations cause glutaric acidemia type IIB, an autosomal recessive disorder affecting mitochondrial energy metabolism. The gene shows low constraint to loss-of-function variation (pLI 0.017), consistent with the recessive inheritance pattern where heterozygous carriers are typically unaffected.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.821 OMIM phenotype
Clinical SummaryETFB
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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 0.02) — 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
0.82LOEUF
pLI 0.017
Z-score 2.01
OE 0.39 (0.200.82)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.42Z-score
OE missense 0.92 (0.811.03)
183 obs / 199.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.39 (0.200.82)
00.351.4
Missense OE0.92 (0.811.03)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 5 / 12.7Missense obs/exp: 183 / 199.8Syn Z: 0.11
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveETFB-related glutaric aciduriaLOFAR

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

DN
0.6842th %ile
GOF
0.5268th %ile
LOF
0.3550th %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

ETFB · 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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