A3GALT2

Chr 1

alpha 1,3-galactosyltransferase 2

This protein synthesizes galactose-alpha(1,3)-galactose groups on glycosphingolipids by transferring galactose from UDP-galactose to acceptor molecules, particularly in the formation of isogloboside 3 (iGb3). Mutations cause congenital anomalies of kidney and urinary tract with or without hearing loss, deafness, or sensorineural hearing loss, inherited in an autosomal recessive pattern. The gene shows tolerance to loss-of-function variants (LOEUF 1.764), primarily affecting kidney and auditory systems.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.76
Clinical SummaryA3GALT2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.76LOEUF
pLI 0.000
Z-score -0.55
OE 1.17 (0.771.76)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.05Z-score
OE missense 0.99 (0.871.12)
170 obs / 171.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.17 (0.771.76)
00.351.4
Missense OE0.99 (0.871.12)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 14 / 11.9Missense obs/exp: 170 / 171.7Syn Z: 0.13

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

A3GALT2 · 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.

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Clinical Literature
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