UBE2E1

Chr 3

ubiquitin conjugating enzyme E2 E1

Also known as: UBCH6

UBE2E1 encodes an E2 ubiquitin-conjugating enzyme that accepts ubiquitin from E1 enzymes and catalyzes its covalent attachment to target proteins, mediating degradation of abnormal and short-lived proteins. The gene is highly constrained against loss-of-function variants (LOEUF 0.57), but specific disease associations and inheritance patterns have not yet been established in the literature. Variants in this gene may potentially cause neurodevelopmental disorders given its essential cellular function and constraint metrics.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.57
Clinical SummaryUBE2E1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.71) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
21 unique Pathogenic / Likely Pathogenic· 9 VUS of 43 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.57LOEUF
pLI 0.705
Z-score 2.35
OE 0.12 (0.040.57)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.99Z-score
OE missense 0.46 (0.360.58)
49 obs / 106.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.12 (0.040.57)
00.351.4
Missense OE0.46 (0.360.58)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 1 / 8.3Missense obs/exp: 49 / 106.9Syn Z: 0.52

ClinVar Variant Classifications

43 submitted variants in ClinVar

Classification Summary

Pathogenic21
VUS9
Likely Benign1
Benign1
21
Pathogenic
9
VUS
1
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
21
0
21
Likely Pathogenic
0
0
0
0
0
VUS
0
8
1
0
9
Likely Benign
0
0
0
1
1
Benign
0
0
0
1
1
Total0822232

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

View in ClinVar →

Protein Context — Lollipop Plot

UBE2E1 · 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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