TBC1D7

Chr 6AR

TBC1 domain family member 7

Also known as: MGCPH, PIG51, TBC7

This protein functions as a non-catalytic component of the TSC-TBC complex that acts as a GTPase-activating protein for RHEB, thereby negatively regulating mTORC1 signaling in response to nutrient availability. Mutations cause autosomal recessive macrocephaly/megalencephaly syndrome. The gene shows low constraint to loss-of-function variation (pLI near zero), consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 1.611 OMIM phenotype
Clinical SummaryTBC1D7
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
1.61LOEUF
pLI 0.000
Z-score -0.29
OE 1.08 (0.741.61)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.14Z-score
OE missense 0.97 (0.851.10)
168 obs / 173.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.08 (0.741.61)
00.351.4
Missense OE0.97 (0.851.10)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 17 / 15.8Missense obs/exp: 168 / 173.4Syn Z: -0.38

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TBC1D7 · 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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