ELOVL5

Chr 6AD

ELOVL fatty acid elongase 5

Also known as: HELO1, SCA38, dJ483K16.1

This protein catalyzes the rate-limiting step in long-chain polyunsaturated fatty acid elongation, specifically acting on polyunsaturated acyl-CoA substrates to produce very long-chain fatty acids that serve as precursors for membrane lipids and lipid mediators. Mutations cause spinocerebellar ataxia 38, inherited in an autosomal dominant pattern. The gene is highly constrained against loss-of-function variants, indicating that protein function is critical for normal cellular processes.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ADLOEUF 0.351 OMIM phenotype
Clinical SummaryELOVL5
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.93). One damaged copy is likely sufficient to cause disease.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.35LOEUF
pLI 0.933
Z-score 3.74
OE 0.14 (0.060.35)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.52Z-score
OE missense 0.69 (0.590.80)
127 obs / 185.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.060.35)
00.351.4
Missense OE0.69 (0.590.80)
00.61.4
Synonymous OE0.77
01.21.6
LoF obs/exp: 3 / 21.8Missense obs/exp: 127 / 185.0Syn Z: 1.54

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ELOVL5 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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