SMCR2

Chr 17

Smith-Magenis syndrome chromosome region, candidate 2

Also known as: TCONS_00025215

SMCR2 encodes a protein that functions as a transcriptional regulator involved in chromatin organization and gene expression control. Mutations cause autosomal recessive intellectual disability with seizures and dysmorphic features, typically presenting in early childhood. The gene shows high constraint against loss-of-function variants, indicating that complete loss of protein function is likely pathogenic.

Clinical SummarySMCR2
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ClinVar Variants
64 unique Pathogenic / Likely Pathogenic· 2 VUS of 66 total submissions

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

66 submitted variants in ClinVar

Classification Summary

Pathogenic64
VUS2
64
Pathogenic
2
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
64
Likely Pathogenic
0
VUS
2
Likely Benign
0
Benign
0
Total66

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SMCR2 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found