ENO3

Chr 17AR

enolase 3

Also known as: GSD13, MSE

This gene encodes one of the three enolase isoenzymes found in mammals. This isoenzyme is found in skeletal muscle cells in the adult where it may play a role in muscle development and regeneration. A switch from alpha enolase to beta enolase occurs in muscle tissue during development in rodents. Mutations in this gene have be associated glycogen storage disease. Alternatively spliced transcript variants encoding different isoforms have been described.[provided by RefSeq, Jul 2010]

OMIMResearchGenerating clinical summary…
DNmechanismARLOEUF 1.691 OMIM phenotype
Clinical SummaryENO3
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?
1.69LOEUF
pLI 0.000
Z-score -0.96
OE 1.22 (0.901.69)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.93Z-score
OE missense 1.16 (1.061.28)
292 obs / 250.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.22 (0.901.69)
00.351.4
Missense OE?1.16 (1.061.28)
00.61.4
Synonymous OE?1.07
01.21.6
LoF obs/exp: 26 / 21.2Missense obs/exp: 292 / 250.8Syn Z: -0.53

This gene — mechanism propensity

DN
0.6938th %ile
GOF
0.5071th %ile
LOF
0.4136th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ENO3 · 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.

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