CD2BP2

Chr 16

CD2 cytoplasmic tail binding protein 2

Also known as: FWP010, LIN1, PPP1R59, Snu40, U5-52K

The protein functions as a component of the U5 small nuclear ribonucleoprotein complex involved in pre-mRNA splicing and also regulates T lymphocyte activation by binding to the CD2 surface antigen. Mutations cause autosomal recessive developmental and epileptic encephalopathy with onset in infancy, characterized by severe intellectual disability, refractory seizures, and progressive microcephaly. The gene shows tolerance to loss-of-function variants with a low constraint score.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.04
Clinical SummaryCD2BP2
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
1.04LOEUF
pLI 0.000
Z-score 1.46
OE 0.61 (0.381.04)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.56Z-score
OE missense 1.11 (0.991.24)
233 obs / 210.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.61 (0.381.04)
00.351.4
Missense OE1.11 (0.991.24)
00.61.4
Synonymous OE1.40
01.21.6
LoF obs/exp: 10 / 16.4Missense obs/exp: 233 / 210.2Syn Z: -2.88
DN
0.6454th %ile
GOF
0.4973th %ile
LOF
0.3452th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

CD2BP2 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →