SCRT1

Chr 8

scratch family transcriptional repressor 1

Also known as: SCRT, ZNF898

This gene encodes a C2H2-type zinc finger transcriptional repressor that binds to E-box motifs and modulates basic helix-loop-helix transcription factors critical for neuronal differentiation. Mutations cause neurodevelopmental disorder with or without anomalies of the brain, eye, or heart, which follows an autosomal dominant inheritance pattern. The gene shows tolerance to loss-of-function variants, suggesting the disorder may involve complex mutational mechanisms.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
58
P/LP submissions
0%
P/LP missense
0.88
LOEUF
Mechanism
Clinical SummarySCRT1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.28) despite low pLI — interpret in context.
📋
ClinVar Variants
57 unique Pathogenic / Likely Pathogenic· 51 VUS of 112 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.88LOEUF
pLI 0.222
Z-score 1.78
OE 0.28 (0.110.88)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
2.43Z-score
OE missense 0.45 (0.370.55)
68 obs / 152.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.28 (0.110.88)
00.351.4
Missense OE0.45 (0.370.55)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 2 / 7.1Missense obs/exp: 68 / 152.5Syn Z: 0.24

ClinVar Variant Classifications

112 submitted variants in ClinVar

Classification Summary

Pathogenic52
Likely Pathogenic5
VUS51
Likely Benign2
Benign1
52
Pathogenic
5
Likely Pathogenic
51
VUS
2
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
52
0
52
Likely Pathogenic
0
0
5
0
5
VUS
0
38
13
0
51
Likely Benign
0
0
1
1
2
Benign
0
0
1
0
1
Total038721111

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SCRT1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗