EPM2AIP1

Chr 3

EPM2A interacting protein 1

EPM2AIP1 encodes a protein that binds to laforin (the EPM2A gene product), though its specific function remains unknown. Mutations cause autosomal recessive progressive myoclonus epilepsy with adolescent onset. The gene is highly constrained against loss-of-function variants, suggesting it plays an important role in neuronal function.

OMIMResearchSummary from RefSeq
LOEUF 0.45
Clinical SummaryEPM2AIP1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.60) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
32 unique Pathogenic / Likely Pathogenic· 75 VUS of 117 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.45LOEUF
pLI 0.600
Z-score 3.35
OE 0.20 (0.100.45)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.44Z-score
OE missense 0.77 (0.700.86)
251 obs / 324.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.20 (0.100.45)
00.351.4
Missense OE0.77 (0.700.86)
00.61.4
Synonymous OE1.18
01.21.6
LoF obs/exp: 4 / 20.3Missense obs/exp: 251 / 324.2Syn Z: -1.60

ClinVar Variant Classifications

117 submitted variants in ClinVar

Classification Summary

Pathogenic31
Likely Pathogenic1
VUS75
Likely Benign6
Benign4
31
Pathogenic
1
Likely Pathogenic
75
VUS
6
Likely Benign
4
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
31
0
31
Likely Pathogenic
0
0
1
0
1
VUS
0
65
10
0
75
Likely Benign
0
3
2
1
6
Benign
0
0
2
2
4
Total068463117

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

EPM2AIP1 · 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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