CCNQ

Chr XXLD

cyclin Q

Also known as: CycM, FAM58A

Mutations in this gene have been shown to cause an X-linked dominant STAR syndrome that typically manifests syndactyly, telecanthus and anogenital and renal malformations. The protein encoded by this gene contains a cyclin-box-fold domain which suggests it may have a role in controlling nuclear cell division cycles. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Oct 2008]

Primary Disease Associations & Inheritance

STAR syndromeMIM #300707
XLD
UniProtToe syndactyly, telecanthus, and anogenital and renal malformations
203
ClinVar variants
118
Pathogenic / LP
pLI score
0
Active trials
Clinical SummaryCCNQ
🧬
Gene-Disease Validity (ClinGen)
syndactyly-telecanthus-anogenital and renal malformations syndrome · XLModerate

Moderate evidence — consider for supplementary testing

📋
ClinVar Variants
118 Pathogenic / Likely Pathogenic· 38 VUS of 203 total submissions
Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

203 submitted variants in ClinVar

Classification Summary

Pathogenic115
Likely Pathogenic3
VUS38
Likely Benign21
Benign26
115
Pathogenic
3
Likely Pathogenic
38
VUS
21
Likely Benign
26
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
0
113
0
115
Likely Pathogenic
0
0
3
0
3
VUS
1
19
17
1
38
Likely Benign
0
5
4
12
21
Benign
3
2
14
7
26
Total62615120203

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

CCNQ · protein map & ClinVar variants

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

Gene2Phenotype Curations

CCNQ-related STAR syndrome

definitive
Monoallelic X HeterozygousLoss Of FunctionAbsent Gene Product
Dev. DisordersSkeletal
G2P ↗

Gene2Phenotype curations · DECIPHER consortium patient cohort (public variants) · deciphergenomics.org

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

CYCLIN Q; CCNQ
MIM #300708 · *

STAR syndrome

MIM #300707

Molecular basis of disorder known

X-linked dominant
Clinical Literature
Landmark / reviewRecent case evidence
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →