TMEM107

Chr 17AR

transmembrane protein 107

Also known as: GRVS638, JBTS29, MKS13, PRO1268

This gene encodes a transmembrane protein and component of the primary cilia transition zone. The encoded protein regulates ciliogenesis and ciliary protein composition. Human fibroblasts expressing a mutant allele of this gene exhibit reduced numbers of cilia, altered cilia length, and impaired sonic hedgehog signaling. In human patients, different mutations in this gene cause different ciliopathies, including Meckel-Gruber syndrome and orofaciodigital syndrome. [provided by RefSeq, May 2017]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

?Joubert syndrome 29MIM #617562
AR
Meckel syndrome 13MIM #617562
AR
Orofaciodigital syndrome XVIMIM #617563
AR
0
Active trials
6
Pubs (1 yr)
P/LP submissions
P/LP missense
1.90
LOEUF
Multiple*
Mechanism· predicted
📖
GeneReview available — TMEM107
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.90LOEUF
pLI 0.000
Z-score -0.91
OE 1.36 (0.821.90)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
1.09Z-score
OE missense 0.67 (0.540.83)
58 obs / 86.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?1.36 (0.821.90)
00.351.4
Missense OE?0.67 (0.540.83)
00.61.4
Synonymous OE?0.86
01.21.6
LoF obs/exp: 10 / 7.3Missense obs/exp: 58 / 86.6Syn Z: 0.68

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TMEM107 · 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.

Search ClinicalTrials.gov →