CCNF

Chr 16AD

cyclin F

Also known as: FBX1, FBXO1, FTDALS5

This protein functions as a substrate recognition component of SCF E3 ubiquitin ligase complexes that target cell cycle regulatory proteins for degradation, including CP110, RRM2, and CDC6. Mutations cause frontotemporal dementia and/or amyotrophic lateral sclerosis 5 with autosomal dominant inheritance. The gene shows significant constraint against loss-of-function variants (LOEUF 0.419), indicating that such variants are likely pathogenic.

OMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismADLOEUF 0.421 OMIM phenotype
Clinical SummaryCCNF
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Gene-Disease Validity (ClinGen)
frontotemporal dementia and/or amyotrophic lateral sclerosis 5 · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.25) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.42LOEUF
pLI 0.127
Z-score 4.44
OE 0.25 (0.150.42)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.74Z-score
OE missense 0.90 (0.830.98)
424 obs / 469.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.25 (0.150.42)
00.351.4
Missense OE0.90 (0.830.98)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 10 / 40.5Missense obs/exp: 424 / 469.2Syn Z: 0.43
DN
0.5279th %ile
GOF
0.7126th %ile
LOF
0.3940th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CCNF · 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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