LSM3

Chr 3

LSM3 homolog, U6 small nuclear RNA and mRNA degradation associated

Also known as: SMX4, USS2, YLR438C

LSM3 encodes a component of the LSM2-8 complex that binds to U6 snRNA and functions in pre-mRNA splicing as part of the U4/U6-U5 tri-snRNP complex and precatalytic spliceosome. The gene shows minimal constraint against loss-of-function variants, and no established human disease associations have been reported. Child neurologists should be cautious when interpreting variants in this gene given the lack of clear phenotype-genotype correlations in the literature.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.77
Clinical SummaryLSM3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
27 unique Pathogenic / Likely Pathogenic· 14 VUS of 47 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.77LOEUF
pLI 0.000
Z-score -0.14
OE 1.05 (0.611.77)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.40Z-score
OE missense 0.85 (0.681.08)
50 obs / 58.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.05 (0.611.77)
00.351.4
Missense OE0.85 (0.681.08)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 8 / 7.6Missense obs/exp: 50 / 58.6Syn Z: 0.04

ClinVar Variant Classifications

47 submitted variants in ClinVar

Classification Summary

Pathogenic26
Likely Pathogenic1
VUS14
Likely Benign1
Benign2
26
Pathogenic
1
Likely Pathogenic
14
VUS
1
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
26
0
26
Likely Pathogenic
0
0
1
0
1
VUS
0
14
0
0
14
Likely Benign
0
0
1
0
1
Benign
0
0
1
1
2
Total01429144

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

View in ClinVar →

Protein Context — Lollipop Plot

LSM3 · 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 ↗