ENTR1

Chr 9

endosome associated trafficking regulator 1

Also known as: NY-CO-3, SDCCAG3, SDDAG3

The protein functions in endosome-to-plasma membrane trafficking and recycling of cargo proteins, regulation of cytokinesis, and formation of primary cilia including localization of proteins like PKD2 to cilia. Mutations cause autosomal recessive neurodevelopmental disorders with intellectual disability, seizures, and movement abnormalities. This gene is not highly constrained against loss-of-function variants.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.35
Clinical SummaryENTR1
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
1.35LOEUF
pLI 0.000
Z-score 0.29
OE 0.93 (0.661.35)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.11Z-score
OE missense 1.19 (1.091.31)
309 obs / 258.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.93 (0.661.35)
00.351.4
Missense OE1.19 (1.091.31)
00.61.4
Synonymous OE1.35
01.21.6
LoF obs/exp: 20 / 21.5Missense obs/exp: 309 / 258.8Syn Z: -3.04
DN
0.6840th %ile
GOF
0.5661th %ile
LOF
0.4234th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ENTR1 · 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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