TBC1D8B

Chr XX-linked

TBC1 domain family member 8B

Also known as: GRAMD8B, NPHS20

This gene encodes a protein with a TBC (Tre-2/Bub2/CDC16) domain. Some mammalian proteins with this domain have been shown to function as Rab-GAPs by binding to specific Rab proteins and affecting their GTPase activity. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2011]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Nephrotic syndrome, type 20MIM #301028
X-linked

Clinical highlights

Gene-disease validity (ClinGen)
nephrotic syndrome, type 20 · XLModerateconsider for supplementary testing
0
Active trials
1
Pubs (1 yr)
P/LP submissions
P/LP missense
0.74
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.74LOEUF
pLI 0.000
Z-score 2.83
OE 0.51 (0.360.74)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.06Z-score
OE missense 0.99 (0.911.08)
372 obs / 375.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.51 (0.360.74)
00.351.4
Missense OE?0.99 (0.911.08)
00.61.4
Synonymous OE?0.91
01.21.6
LoF obs/exp: 20 / 39.1Missense obs/exp: 372 / 375.1Syn Z: 0.84

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TBC1D8B · 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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