TBC1D7

Chr 6

TBC1 domain family member 7

Also known as: MGCPH, PIG51, TBC7

This gene encodes a member of the TBC-domain containing protein family. The encoded protein functions as a subunit of the tuberous sclerosis TSC1-TSC2 complex which plays a role in the regulation of cellular growth and differentiation. Mutations in this gene have been associated with autosomal recessive megalencephaly. Alternative splicing results in multiple transcript variants. Naturally occurring readthrough transcription occurs between this locus and downstream LOC100130357. [provided by RefSeq, Jan 2016]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMacrocephaly/megalencephaly syndrome, autosomal recessive
0
Active trials
9
Pubs (1 yr)
P/LP submissions
P/LP missense
1.61
LOEUF
Mechanism
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

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 OE?1.08 (0.741.61)
00.351.4
Missense OE?0.97 (0.851.10)
00.61.4
Synonymous OE?1.06
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.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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