UBR7

Chr 14AR

ubiquitin protein ligase E3 component n-recognin 7

Also known as: C14orf130, LICAS

The protein is an E3 ubiquitin ligase that recognizes and targets proteins with destabilizing N-terminal residues for ubiquitination and proteasomal degradation as part of the N-end rule pathway. Biallelic mutations cause Li-Campeau syndrome, an autosomal recessive disorder. The gene shows significant constraint against loss-of-function variants (LOEUF 0.447), indicating intolerance to protein disruption.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.451 OMIM phenotype
Clinical SummaryUBR7
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.21) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.45LOEUF
pLI 0.494
Z-score 3.54
OE 0.21 (0.110.45)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.85Z-score
OE missense 0.65 (0.570.75)
145 obs / 222.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.21 (0.110.45)
00.351.4
Missense OE0.65 (0.570.75)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 5 / 23.5Missense obs/exp: 145 / 222.6Syn Z: 1.04

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

UBR7 · 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 →