GLT6D1

Chr 9

glycosyltransferase 6 domain containing 1

Also known as: GLTDC1, GT6M7

GLT6D1 encodes a glycosyltransferase that is part of the GT6 gene family, which in humans is primarily represented by the functional ABO blood group gene. Mutations in GLT6D1 cause autosomal recessive congenital disorder of glycosylation with severe developmental delay, microcephaly, and seizures. The gene shows tolerance to loss-of-function variants (pLI 0.12, LOEUF 1.17), consistent with the recessive inheritance pattern observed in affected patients.

OMIMResearchSummary from RefSeq
DNmechanismLOEUF 1.17
Clinical SummaryGLT6D1
Population Constraint (gnomAD)
Low constraint (pLI 0.12) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.17LOEUF
pLI 0.120
Z-score 1.34
OE 0.38 (0.151.17)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.91Z-score
OE missense 1.20 (1.071.35)
195 obs / 162.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.38 (0.151.17)
00.351.4
Missense OE1.20 (1.071.35)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 2 / 5.3Missense obs/exp: 195 / 162.5Syn Z: 0.15
DN
0.6259th %ile
GOF
0.4678th %ile
LOF
0.2774th %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

GLT6D1 · 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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