FBXO10

Chr 9

F-box protein 10

Also known as: FBX10, PRMT11

FBXO10 encodes a substrate-recognition component of SCF E3 ubiquitin ligase complexes that mediates the ubiquitination and degradation of BCL2, RAGE, and HGAL proteins, thereby regulating apoptosis and B-cell receptor signaling. Mutations cause autosomal recessive intellectual disability with variable features including microcephaly, seizures, and developmental delays. The gene is highly constrained against loss-of-function variants (LOEUF 0.648), suggesting intolerance to protein disruption.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.65
Clinical SummaryFBXO10
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 3.25
OE 0.43 (0.290.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.89Z-score
OE missense 0.78 (0.720.84)
466 obs / 595.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.43 (0.290.65)
00.351.4
Missense OE0.78 (0.720.84)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 16 / 37.5Missense obs/exp: 466 / 595.9Syn Z: 1.85

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FBXO10 · 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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