CSRNP1

Chr 3

cysteine and serine rich nuclear protein 1

Also known as: AXUD1, CSRNP-1, FAM130B, TAIP-3, URAX1

This gene encodes a nuclear transcription factor that binds to specific DNA sequences and activates transcription, and may function in apoptosis regulation. Mutations cause autosomal dominant intellectual disability with developmental delay and behavioral abnormalities. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.525), suggesting some intolerance to complete protein loss.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.53
Clinical SummaryCSRNP1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.23) despite low pLI — interpret in context.
📋
ClinVar Variants
9 unique Pathogenic / Likely Pathogenic· 95 VUS of 116 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.53LOEUF
pLI 0.343
Z-score 2.98
OE 0.23 (0.110.53)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.29Z-score
OE missense 0.96 (0.881.05)
344 obs / 359.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.23 (0.110.53)
00.351.4
Missense OE0.96 (0.881.05)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 4 / 17.4Missense obs/exp: 344 / 359.5Syn Z: 0.46

ClinVar Variant Classifications

116 submitted variants in ClinVar

Classification Summary

Pathogenic8
Likely Pathogenic1
VUS95
Likely Benign6
8
Pathogenic
1
Likely Pathogenic
95
VUS
6
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
8
0
8
Likely Pathogenic
0
0
1
0
1
VUS
0
95
0
0
95
Likely Benign
0
6
0
0
6
Benign
0
0
0
0
0
Total010190110

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

View in ClinVar →

Protein Context — Lollipop Plot

CSRNP1 · 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.

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