UBE2R2

Chr 9

ubiquitin conjugating enzyme E2 R2

Also known as: CDC34B, E2-CDC34B, UBC3B

This gene encodes an E2 ubiquitin-conjugating enzyme that transfers ubiquitin from E1 activating enzymes to target proteins via E3 ligase complexes, participating in protein degradation pathways including beta-catenin regulation. The gene is highly constrained against loss-of-function variants (pLI 0.98, LOEUF 0.26), suggesting mutations would likely cause severe disease, though specific associated disorders have not yet been established in the literature. Clinical phenotypes and inheritance patterns remain to be characterized as this gene emerges in research studies.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.26
Clinical SummaryUBE2R2
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
69 unique Pathogenic / Likely Pathogenic· 17 VUS of 95 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.26LOEUF
pLI 0.978
Z-score 3.17
OE 0.00 (0.000.26)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.78Z-score
OE missense 0.31 (0.240.40)
40 obs / 129.0 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.00 (0.000.26)
00.351.4
Missense OE0.31 (0.240.40)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 0 / 11.7Missense obs/exp: 40 / 129.0Syn Z: 0.18
DN
0.3594th %ile
GOF
0.4973th %ile
LOF
0.68top 25%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.26

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

95 submitted variants in ClinVar

Classification Summary

Pathogenic61
Likely Pathogenic8
VUS17
Likely Benign2
61
Pathogenic
8
Likely Pathogenic
17
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
61
Likely Pathogenic
8
VUS
17
Likely Benign
2
Benign
0
Total88

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

UBE2R2 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC