ACAD8

Chr 11

acyl-CoA dehydrogenase family member 8

Also known as: ACAD-8, ARC42, IBDH

This gene encodes a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. The encoded protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine. Defects in this gene are the cause of isobutyryl-CoA dehydrogenase deficiency.[provided by RefSeq, Nov 2009]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtIsobutyryl-CoA dehydrogenase deficiency

Clinical highlights

Gene-disease validity (ClinGen)
isobutyryl-CoA dehydrogenase deficiency · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
9
Pubs (1 yr)
P/LP submissions
P/LP missense
1.27
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.27LOEUF
pLI 0.000
Z-score 0.51
OE 0.89 (0.641.27)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.22Z-score
OE missense 0.96 (0.861.07)
221 obs / 230.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.89 (0.641.27)
00.351.4
Missense OE?0.96 (0.861.07)
00.61.4
Synonymous OE?1.26
01.21.6
LoF obs/exp: 22 / 24.7Missense obs/exp: 221 / 230.5Syn Z: -1.95

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ACAD8 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →