GALNT2

Chr 1AR

polypeptide N-acetylgalactosaminyltransferase 2

Also known as: CDG2T, GalNAc-T2

The protein catalyzes the initial step in O-linked oligosaccharide biosynthesis by transferring N-acetyl-D-galactosamine to serine or threonine residues on target proteins, with particular involvement in immunoglobulin A1 hinge region glycosylation and HDL-C metabolism regulation. Mutations cause congenital disorder of glycosylation type IIt with autosomal recessive inheritance. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.488).

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.491 OMIM phenotype
Clinical SummaryGALNT2
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Gene-Disease Validity (ClinGen)
congenital disorder of glycosylation, type iit · ARStrong

Strong evidence — appropriate for clinical testing

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.49LOEUF
pLI 0.008
Z-score 3.99
OE 0.29 (0.180.49)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.00Z-score
OE missense 0.70 (0.630.78)
243 obs / 348.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.29 (0.180.49)
00.351.4
Missense OE0.70 (0.630.78)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 11 / 37.3Missense obs/exp: 243 / 348.1Syn Z: -0.24
DN
0.6453th %ile
GOF
0.6149th %ile
LOF
0.3356th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GALNT2 · 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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