SCHIP1

Chr 3

schwannomin interacting protein 1

Also known as: SCHIP-1

SCHIP1 enables identical protein binding activity and is predicted to be involved in positive regulation of hippo signaling and various developmental processes including face morphogenesis. Mutations cause autosomal dominant neurodevelopmental disorder with seizures, hypotonia, and brain imaging abnormalities. The gene is highly constrained against loss-of-function variants, consistent with its role in essential developmental pathways.

OMIMResearchSummary from RefSeq
LOFmechanismLOEUF 0.23
Clinical SummarySCHIP1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). 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
0.23LOEUF
pLI 0.987
Z-score 3.36
OE 0.00 (0.000.23)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.90Z-score
OE missense 0.53 (0.430.65)
68 obs / 128.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.000.23)
00.351.4
Missense OE0.53 (0.430.65)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 0 / 13.2Missense obs/exp: 68 / 128.8Syn Z: 0.54
DN
0.4983th %ile
GOF
0.5759th %ile
LOF
0.65top 25%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.23

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SCHIP1 · 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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