TMEM231

Chr 16AR

transmembrane protein 231

Also known as: ALYE870, JBTS20, MKS11, PRO1886

This gene encodes a transmembrane component of the tectonic-like complex that acts as a barrier preventing diffusion of transmembrane proteins between the cilia and plasma membranes, and is required for ciliogenesis and sonic hedgehog signaling. Mutations cause Joubert syndrome and Meckel syndrome, both severe ciliopathies affecting the brain, kidneys, liver, and other organ systems. The gene follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.212 OMIM phenotypes
Clinical SummaryTMEM231
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Gene-Disease Validity (ClinGen)
ciliopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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
1.21LOEUF
pLI 0.000
Z-score 0.93
OE 0.75 (0.481.21)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.41Z-score
OE missense 1.08 (0.971.22)
201 obs / 185.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.75 (0.481.21)
00.351.4
Missense OE1.08 (0.971.22)
00.61.4
Synonymous OE1.10
01.21.6
LoF obs/exp: 12 / 16.0Missense obs/exp: 201 / 185.3Syn Z: -0.68

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TMEM231 · 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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