RBBP8

Chr 18

RB binding protein 8, endonuclease

Also known as: COM1, CTIP, JAWAD, JWDS, RIM, SAE2, SCKL2

The protein encoded by this gene is a ubiquitously expressed nuclear protein. It is found among several proteins that bind directly to retinoblastoma protein, which regulates cell proliferation. This protein complexes with transcriptional co-repressor CTBP. It is also associated with BRCA1 and is thought to modulate the functions of BRCA1 in transcriptional regulation, DNA repair, and/or cell cycle checkpoint control. It is suggested that this gene may itself be a tumor suppressor acting in the same pathway as BRCA1. Three transcript variants encoding two different isoforms have been found for this gene. More transcript variants exist, but their full-length natures have not been determined. [provided by RefSeq, Jul 2008]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSeckel syndrome 2
UniProtJawad syndrome
0
Active trials
6
Pubs (1 yr)
P/LP submissions
P/LP missense
0.75
LOEUF
DN
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.75LOEUF
pLI 0.000
Z-score 2.99
OE 0.54 (0.400.75)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.02Z-score
OE missense 1.00 (0.931.08)
466 obs / 464.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.54 (0.400.75)
00.351.4
Missense OE?1.00 (0.931.08)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 27 / 49.7Missense obs/exp: 466 / 464.8Syn Z: 0.09

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RBBP8 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →