Murine UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase lacking the gamma-subunit retains substantial activity toward acid hydrolases
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Mucolipidosis II-related mutations inhibit the exit from the endoplasmic reticulum and proteolytic cleavage of GlcNAc-1-phosphotransferase precursor protein (GNPTAB)Functions of the alpha, beta, and gamma subunits of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferaseMice lacking mannose 6-phosphate uncovering enzyme activity have a milder phenotype than mice deficient for N-acetylglucosamine-1-phosphotransferase activity.Post-translational modifications of the gamma-subunit affect intracellular trafficking and complex assembly of GlcNAc-1-phosphotransferaseComparative pathology of murine mucolipidosis types II and IIICImproved retroviral vector design results in sustained expression after adult gene therapy in mucopolysaccharidosis I mice.Comparative Analysis of Protein Glycosylation Pathways in Humans and the Fungal Pathogen Candida albicans.Selective yolk deposition and mannose phosphorylation of lysosomal glycosidases in zebrafishNeurologic abnormalities in mouse models of the lysosomal storage disorders mucolipidosis II and mucolipidosis III γ.Latency-associated peptide of transforming growth factor-β1 is not subject to physiological mannose phosphorylationThe lysosomal enzyme receptor protein (LERP) is not essential, but is implicated in lysosomal function in Drosophila melanogaster.Mucolipidosis III GNPTG Missense Mutations Cause Misfolding of the γ Subunit of GlcNAc-1-Phosphotransferase.Strategies for carbohydrate recognition by the mannose 6-phosphate receptors.Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II.Glycosylation- and phosphorylation-dependent intracellular transport of lysosomal hydrolases.Carbohydrate recognition by the mannose-6-phosphate receptorsMannose 6-phosphate receptor homology (MRH) domain-containing lectins in the secretory pathway.Altered Met Receptor Phosphorylation and LRP1 Mediated Uptake in Cells Lacking Carbohydrate-Dependent Lysosomal Targeting.Subunit interactions of the disease-related hexameric GlcNAc-1-phosphotransferase complex.Quantitative Proteome Analysis of Mouse Liver Lysosomes Provides Evidence for Mannose 6-phosphate-independent Targeting Mechanisms of Acid Hydrolases in Mucolipidosis II.Enzyme-specific differences in mannose phosphorylation between GlcNAc-1-phosphotransferase αβ and γ subunit deficient zebrafish support cathepsin proteases as early mediators of mucolipidosis pathology.Characterization and downstream mannose phosphorylation of human recombinant α-L-iduronidase produced in Arabidopsis complex glycan-deficient (cgl) seedsVacuolization of mucolipidosis type II mouse exocrine gland cells represents accumulation of autolysosomes.Proteolytic processing of the gamma-subunit is associated with the failure to form GlcNAc-1-phosphotransferase complexes and mannose 6-phosphate residues on lysosomal enzymes in human macrophages.Disruption of the Man-6-P targeting pathway in mice impairs osteoclast secretory lysosome biogenesis.Enigmatic in vivo GlcNAc-1-phosphotransferase (GNPTG) transcript correction to wild type in two mucolipidosis III gamma siblings homozygous for nonsense mutations.N-glycan structures and downstream mannose-phosphorylation of plant recombinant human alpha-L-iduronidase: toward development of enzyme replacement therapy for mucopolysaccharidosis I.Lysosomal Proteome and Secretome Analysis Identifies Missorted Enzymes and Their Nondegraded Substrates in Mucolipidosis III Mouse Cells
P2860
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P2860
Murine UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase lacking the gamma-subunit retains substantial activity toward acid hydrolases
description
2007 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique Journal of Biological Chemistry
@fr
artículu científicu espublizáu en 2007
@ast
im September 2007 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2007/09/14)
@sk
vědecký článek publikovaný v roce 2007
@cs
wetenschappelijk artikel (gepubliceerd op 2007/09/14)
@nl
наукова стаття, опублікована у вересні 2007
@uk
name
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@ast
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@en
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@nl
type
label
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@ast
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@en
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@nl
prefLabel
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@ast
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@en
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@nl
P2093
P2860
P356
P1476
Murine UDP-GlcNAc:lysosomal en ...... ctivity toward acid hydrolases
@en
P2093
Bobby Joe Payne
Claire M. Gelfman
Peter Vogel
Stuart Kornfeld
Wang-Sik Lee
P2860
P304
27198–27203
P356
10.1074/JBC.M704067200
P407
P577
2007-09-14T00:00:00Z