TBC1D30

Chr 12

TBC1 domain family member 30

The protein acts as a GTPase-activating protein for Rab family proteins and regulates cilium assembly. Mutations cause autosomal recessive intellectual disability with seizures and brain abnormalities, typically presenting in infancy or early childhood. This gene is highly constrained against loss-of-function variants (pLI 0.89, LOEUF 0.35), indicating intolerance to protein disruption.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.35
Clinical SummaryTBC1D30
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.89) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.35LOEUF
pLI 0.889
Z-score 4.41
OE 0.18 (0.100.35)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.70Z-score
OE missense 0.63 (0.560.69)
259 obs / 413.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.18 (0.100.35)
00.351.4
Missense OE0.63 (0.560.69)
00.61.4
Synonymous OE0.76
01.21.6
LoF obs/exp: 6 / 33.6Missense obs/exp: 259 / 413.5Syn Z: 2.47

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TBC1D30 · 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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