UIMC1

Chr 5

ubiquitin interaction motif containing 1

Also known as: RAP80, X2HRIP110

The UIMC1 protein specifically recognizes and binds Lys-63-linked ubiquitinated histones at DNA damage sites, functioning as part of the BRCA1-A complex to recruit DNA repair machinery to double-strand breaks. Mutations cause autosomal recessive intellectual disability with microcephaly and variable features including growth retardation and facial dysmorphism. This gene shows low constraint against loss-of-function variants, consistent with the recessive inheritance pattern observed in affected families.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.65
Clinical SummaryUIMC1
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
0.65LOEUF
pLI 0.000
Z-score 3.27
OE 0.44 (0.300.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.71Z-score
OE missense 0.90 (0.820.98)
340 obs / 379.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.44 (0.300.65)
00.351.4
Missense OE0.90 (0.820.98)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 17 / 39.1Missense obs/exp: 340 / 379.2Syn Z: -0.24

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

UIMC1 · 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 →