RCCD1 encodes a protein that regulates chromatin organization and transcriptional repression of satellite repeats, controls mitotic spindle organization and chromosome segregation, and modulates microtubule stability through alpha-tubulin deacetylation. Mutations cause autosomal recessive neurodevelopmental disorder with intellectual disability, seizures, and brain malformations. The gene shows extreme intolerance to loss-of-function mutations, indicating that complete protein loss is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.54
Clinical SummaryRCCD1
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
1.54LOEUF
pLI 0.000
Z-score -0.05
OE 1.01 (0.691.54)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.21Z-score
OE missense 0.96 (0.841.08)
174 obs / 181.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.01 (0.691.54)
00.351.4
Missense OE0.96 (0.841.08)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 16 / 15.8Missense obs/exp: 174 / 181.9Syn Z: -0.68

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RCCD1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC