DIPK1B

Chr 9

divergent protein kinase domain 1B

Also known as: C9orf136, FAM69B, pp6977

This gene encodes a cysteine-rich transmembrane protein that localizes to the endoplasmic reticulum, though its specific cellular function remains unknown. Mutations cause autosomal recessive intellectual disability with seizures and brain malformations. The gene shows tolerance to loss-of-function variants in the general population.

OMIMResearchSummary from RefSeq
LOEUF 0.72
Clinical SummaryDIPK1B
Population Constraint (gnomAD)
Low constraint (pLI 0.01) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
71 unique Pathogenic / Likely Pathogenic· 118 VUS of 205 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.72LOEUF
pLI 0.012
Z-score 2.37
OE 0.37 (0.200.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.35Z-score
OE missense 1.06 (0.961.17)
300 obs / 283.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.37 (0.200.72)
00.351.4
Missense OE1.06 (0.961.17)
00.61.4
Synonymous OE1.20
01.21.6
LoF obs/exp: 6 / 16.3Missense obs/exp: 300 / 283.4Syn Z: -1.82

ClinVar Variant Classifications

205 submitted variants in ClinVar

Classification Summary

Pathogenic68
Likely Pathogenic3
VUS118
Likely Benign4
Benign1
Conflicting2
68
Pathogenic
3
Likely Pathogenic
118
VUS
4
Likely Benign
1
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
68
0
68
Likely Pathogenic
0
0
3
0
3
VUS
0
110
8
0
118
Likely Benign
0
4
0
0
4
Benign
0
0
1
0
1
Conflicting
2
Total0114800196

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

View in ClinVar →

Protein Context — Lollipop Plot

DIPK1B · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found