SNIP1

Chr 1AR

Smad nuclear interacting protein 1

Also known as: NEDHCS, PML1, PMRED

The SNIP1 protein functions as a transcriptional coactivator that modulates c-Myc, TGF-beta, and NF-kappa-B signaling pathways, and serves as a component of the spliceosome required for pre-mRNA splicing. Biallelic mutations cause autosomal recessive neurodevelopmental disorder with hypotonia, craniofacial abnormalities, and seizures. This condition involves the central nervous system and craniofacial development, with seizures and developmental delays as prominent features.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 0.501 OMIM phenotype
Clinical SummarySNIP1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) despite low pLI — interpret in context.
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GeneReview available — SNIP1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.50LOEUF
pLI 0.422
Z-score 3.10
OE 0.22 (0.110.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.24Z-score
OE missense 0.78 (0.700.88)
201 obs / 256.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.110.50)
00.351.4
Missense OE0.78 (0.700.88)
00.61.4
Synonymous OE0.83
01.21.6
LoF obs/exp: 4 / 18.3Missense obs/exp: 201 / 256.8Syn Z: 1.26

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SNIP1 · 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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