ST8SIA5

Chr 18

ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5

Also known as: SIAT8-E, SIAT8E, ST8SiaV

The protein is a Golgi-localized glycosyltransferase that synthesizes specific gangliosides (GD1c, GT1a, GQ1b, and GT3) from precursor gangliosides. Mutations cause autosomal recessive intellectual disability with seizures and spasticity. The gene shows tolerance to loss-of-function variants in the general population.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
39
P/LP submissions
0%
P/LP missense
0.85
LOEUF
Mechanism
Clinical SummaryST8SIA5
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
38 unique Pathogenic / Likely Pathogenic· 52 VUS of 100 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.85LOEUF
pLI 0.001
Z-score 2.00
OE 0.47 (0.280.85)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.90Z-score
OE missense 0.84 (0.750.94)
205 obs / 244.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.47 (0.280.85)
00.351.4
Missense OE0.84 (0.750.94)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 8 / 16.9Missense obs/exp: 205 / 244.4Syn Z: 0.69

ClinVar Variant Classifications

100 submitted variants in ClinVar

Classification Summary

Pathogenic37
Likely Pathogenic1
VUS52
Likely Benign1
Benign3
37
Pathogenic
1
Likely Pathogenic
52
VUS
1
Likely Benign
3
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
37
0
37
Likely Pathogenic
0
0
1
0
1
VUS
0
48
4
0
52
Likely Benign
0
0
1
0
1
Benign
0
1
0
2
3
Total04943294

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

View in ClinVar →

Protein Context — Lollipop Plot

ST8SIA5 · 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
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found