CSNK2B

Chr 6AD

casein kinase 2 beta

Also known as: CK II beta, CK2B, CK2N, CSK2B, Ckb1, Ckb2, G5A, POBINDS

The beta subunit of casein kinase II serves regulatory functions within this tetrameric protein kinase that controls metabolic pathways, signal transduction, transcription, translation, and replication. Mutations cause Poirier-Bienvenu neurodevelopmental syndrome through an autosomal dominant inheritance pattern. The pathogenic mechanism involves loss of function, consistent with the protein's essential regulatory role in multiple cellular processes.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismADLOEUF 0.381 OMIM phenotype
Clinical SummaryCSNK2B
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Gene-Disease Validity (ClinGen)
Poirier-Bienvenu neurodevelopmental syndrome · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.92). One damaged copy is likely sufficient to cause disease.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.38LOEUF
pLI 0.917
Z-score 3.00
OE 0.08 (0.030.38)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
3.13Z-score
OE missense 0.21 (0.160.30)
27 obs / 125.8 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.08 (0.030.38)
00.351.4
Missense OE0.21 (0.160.30)
00.61.4
Synonymous OE0.83
01.21.6
LoF obs/exp: 1 / 12.4Missense obs/exp: 27 / 125.8Syn Z: 0.91
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveCSNK2B-related Poirier-Bienvenu neurodevelopmental syndromeLOFAD
DN
0.3594th %ile
GOF
0.3986th %ile
LOF
0.66top 25%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · 1 literature citation · LOEUF 0.38

Literature Evidence

LOFOur results indicate that CSNK2B haploinsufficiency plays a crucial role in the development of the CSNK2B deficiency phenotype.PMID:33758130

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CSNK2B · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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