UDP-N-acetylglucosamine:glycoprotein N-acetylglucosamine-1-phosphotransferase. Proposed enzyme for the phosphorylation of the high mannose oligosaccharide units of lysosomal enzymes
about
Analyses of disease-related GNPTAB mutations define a novel GlcNAc-1-phosphotransferase interaction domain and an alternative site-1 protease cleavage siteDemonstration of the heterozygous state for I-cell disease and pseudo-Hurler polydystrophy by assay of N-acetylglucosaminylphosphotransferase in white blood cells and fibroblastsPost-translational modifications of the gamma-subunit affect intracellular trafficking and complex assembly of GlcNAc-1-phosphotransferaseMucolipidosis II and III. The genetic relationships between two disorders of lysosomal enzyme biosynthesis.The overexpressed human 46-kDa mannose 6-phosphate receptor mediates endocytosis and sorting of beta-glucuronidase.Mucolipidosis II (I-cell disease) and mucolipidosis IIIA (classical pseudo-hurler polydystrophy) are caused by mutations in the GlcNAc-phosphotransferase alpha / beta -subunits precursor gene.Pitfalls in the prenatal diagnosis of mucolipidosis II alpha/beta: A case report.High resolution crystal structure of human β-glucuronidase reveals structural basis of lysosome targeting.Glucosidase II and MRH-domain containing proteins in the secretory pathway.Biosynthesis of lysosomal hydrolases: their synthesis in bound polysomes and the role of co- and post-translational processing in determining their subcellular distribution.Lysosomal enzyme oligosaccharide phosphorylation in mouse lymphoma cells: specificity and kinetics of binding to the mannose 6-phosphate receptor in vivoLysosomal enzyme trafficking in mannose 6-phosphate receptor-positive mouse L-cells: demonstration of a steady state accumulation of phosphorylated acid hydrolasesMannose 6-phosphate receptor-mediated endocytosis of acid hydrolases: internalization of beta-glucuronidase is accompanied by a limited dephosphorylation.Postendocytic maturation of acid hydrolases: evidence of prelysosomal processing.Expression of human cathepsin D in Xenopus oocytes: phosphorylation and intracellular targetingRenin, a secretory glycoprotein, acquires phosphomannosyl residuesUteroferrin has N-asparagine-linked high-mannose-type oligosaccharides that contain mannose 6-phosphate.Synthesis of beta-hexosaminidase in cell-free translation and in intact fibroblasts: an insoluble precursor alpha chain in a rare form of Tay-Sachs disease.Mucolipidosis III is genetically heterogeneous.Identification of a variant of mucolipidosis III (pseudo-Hurler polydystrophy): a catalytically active N-acetylglucosaminylphosphotransferase that fails to phosphorylate lysosomal enzymes.A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth.Mannose 6-phosphate receptor targeting and its applications in human diseases.N-/O-glycosylation analysis of human FVIIa produced in the milk of transgenic rabbitsGlycan microarray analysis of P-type lectins reveals distinct phosphomannose glycan recognitionIdentification and characterization of cells deficient in the mannose 6-phosphate receptor: evidence for an alternate pathway for lysosomal enzyme targeting.A novel intermediate mucolipidosis II/IIIαβ caused by GNPTAB mutation in the cytosolic N-terminal domain.Lysosomal storage disorders: molecular basis and laboratory testing.Mannose 6-phosphate receptor homology (MRH) domain-containing lectins in the secretory pathway.Trafficking of lysosomal enzymes in normal and disease states.Transport and processing of beta-hexosaminidase in normal and mucolipidosis-II cultured fibroblasts. Effect of monensin and nigericin.Matriglycan: a novel polysaccharide that links dystroglycan to the basement membrane.TGN exit of the cation-independent mannose 6-phosphate receptor does not require acid hydrolase binding.Biological roles of glycans.Quantitative Proteome Analysis of Mouse Liver Lysosomes Provides Evidence for Mannose 6-phosphate-independent Targeting Mechanisms of Acid Hydrolases in Mucolipidosis II.Transit of alpha-mannosidase during its maturation in Dictyostelium discoideum.Human lysosomal alpha-glucosidase: functional characterization of the glycosylation sites.Targeting of beta-glucuronidase to lysosomes in mannose 6-phosphate receptor-deficient MOPC 315 cells.Impaired clearance of free cystine from lysosome-enriched granular fractions of I-cell-disease fibroblasts.Properties of N-acetylglucosamine 1-phosphotransferase from human lymphoblasts.Increased N-acetylglucosamine-1-phosphotransferase activity in sera from patients with leukemia.
P2860
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P2860
UDP-N-acetylglucosamine:glycoprotein N-acetylglucosamine-1-phosphotransferase. Proposed enzyme for the phosphorylation of the high mannose oligosaccharide units of lysosomal enzymes
description
1981 nî lūn-bûn
@nan
1981 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
1981 թվականի մայիսին հրատարակված գիտական հոդված
@hy
1981年の論文
@ja
1981年論文
@yue
1981年論文
@zh-hant
1981年論文
@zh-hk
1981年論文
@zh-mo
1981年論文
@zh-tw
1981年论文
@wuu
name
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@ast
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@en
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@nl
type
label
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@ast
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@en
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@nl
prefLabel
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@ast
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@en
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@nl
P1476
UDP-N-acetylglucosamine:glycop ...... ide units of lysosomal enzymes
@en
P2093
Kornfeld S
P304
P407
P577
1981-05-01T00:00:00Z