GNPTG Chromosome 16

N-acetylglucosamine-1-phosphate transferase subunit gamma
130 variants 130 Health Risk

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What This Gene Does
This gene encodes the gamma sunbunit of the N-acetylglucosamine-1-phosphotransferase complex. This hexameric complex, composed of alpha, beta and gamma subunits, catalyzes the first step in synthesis of a mannose 6-phosphate lysosomal recognition marker. This enzyme complex is necessary for targeting of lysosomal hydrolases to the lysosome. Mutations in the gene encoding the gamma subunit have been associated with mucolipidosis IIIC, also known as mucolipidosis III gamma.[provided by RefSeq, Feb 2010]
Gene Info
Gene Group
MRH domain containing
Locus Type
gene with protein product
Location
16p13.3
Ensembl
ENSG00000090581
Associated Conditions (10)
GNPTG-mucolipidosis
Inborn genetic diseases
GNPTG-related disorder
Retinal dystrophy
Malignant tumor of urinary bladder
Mucolipidosis
Familial cancer of breast
Melanoma
Nonpapillary renal cell carcinoma
Rod-cone dystrophy
Key Variants
All Variants (130)
RSID Category Clinical Significance Conditions
RS368983807 Health Risk Likely pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS749952316 Health Risk Likely pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS771844889 Health Risk Likely pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS772287534 Health Risk Likely pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS775359476 Health Risk Likely pathogenic
RS779597308 Health Risk Likely pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS988175540 Health Risk Likely pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS112850896 Health Risk Pathogenic GNPTG-mucolipidosis, Melanoma, Nonpapillary renal cell carcinoma
RS1195785709 Health Risk Pathogenic
RS1260510628 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS1291093292 Health Risk Pathogenic
RS1291377834 Health Risk Pathogenic
RS1297654273 Health Risk Pathogenic
RS1321245057 Health Risk Pathogenic
RS1336176104 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS137852884 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS1394867404 Health Risk Pathogenic
RS1451538466 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS1555451643 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS1555452081 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302847 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302848 Health Risk Pathogenic GNPTG-mucolipidosis, Retinal dystrophy, GNPTG-mucolipidosis
RS193302852 Health Risk Pathogenic
RS193302853 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302854 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302855 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302856 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302858 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS193302859 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS2034895916 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS2034919426 Health Risk Pathogenic
RS2034926616 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS2034941684 Health Risk Pathogenic
RS2141633130 Health Risk Pathogenic
RS2141862610 Health Risk Pathogenic
RS2141862900 Health Risk Pathogenic
RS2141863595 Health Risk Pathogenic
RS2141863669 Health Risk Pathogenic
RS2141863683 Health Risk Pathogenic
RS2141864067 Health Risk Pathogenic
RS2141864091 Health Risk Pathogenic
RS2141864128 Health Risk Pathogenic
RS2141864300 Health Risk Pathogenic
RS2141864418 Health Risk Pathogenic GNPTG-mucolipidosis, GNPTG-mucolipidosis
RS2548185457 Health Risk Pathogenic
RS2548185516 Health Risk Pathogenic
RS2548185517 Health Risk Pathogenic
RS2548185737 Health Risk Pathogenic
RS2548185743 Health Risk Pathogenic
RS2548185876 Health Risk Pathogenic
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