GNPTG

Chr 16AR

N-acetylglucosamine-1-phosphate transferase subunit gamma

Non-catalytic subunit of the N-acetylglucosamine-1-phosphotransferase complex, an enzyme that catalyzes the formation of mannose 6-phosphate (M6P) markers on high mannose type oligosaccharides in the Golgi apparatus. Binds and presents the high mannose glycans of the acceptor to the catalytic alpha and beta subunits (GNPTAB). Enhances the rate of N-acetylglucosamine-1-phosphate transfer to the oligosaccharides of acid hydrolase acceptors

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 1.221 OMIM phenotype
Clinical SummaryGNPTG
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Gene-Disease Validity (ClinGen)
GNPTG-mucolipidosis · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

ncbi: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.22LOEUF
pLI 0.000
Z-score 0.85
OE 0.79 (0.531.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-2.06Z-score
OE missense 1.44 (1.301.60)
253 obs / 176.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.79 (0.531.22)
00.351.4
Missense OE?1.44 (1.301.60)
00.61.4
Synonymous OE?1.70
01.21.6
LoF obs/exp: 15 / 19.0Missense obs/exp: 253 / 176.0Syn Z: -4.86
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveGNPTG-related mucolipidosis type III complementation group CLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6452th %ile
GOF
0.4973th %ile
LOF
0.3068th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GNPTG · 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.

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