TMEM204

Chr 16

transmembrane protein 204

Also known as: C16orf30, CLP24

The TMEM204 protein regulates paracellular permeability and participates in cell-cell interactions through adherens junctions. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly, which typically presents in infancy with seizures and developmental delays. This gene is highly constrained against loss-of-function variants, indicating its critical importance for normal cellular function.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.38
Clinical SummaryTMEM204
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.91). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.38LOEUF
pLI 0.913
Z-score 2.60
OE 0.00 (0.000.38)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.99Z-score
OE missense 0.78 (0.670.90)
124 obs / 159.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.38)
00.351.4
Missense OE0.78 (0.670.90)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 0 / 7.8Missense obs/exp: 124 / 159.3Syn Z: -0.28

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TMEM204 · 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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