Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility. - Wikidata - wikimedia - TriplyDB

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

http://www.wikidata.org/entity/Q42196992

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Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-acti Human α-l-iduronidase uses its own N -glycan as a substrate-binding and catalytic module Regulation of lysosomal ion homeostasis by channels and transporters Fabry disease: preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice.Structural determinants of protein folding.Glycosylation of therapeutic proteins: an effective strategy to optimize efficacy Fabry disease: novel alpha-galactosidase A 3'-terminal mutations result in multiple transcripts due to aberrant 3'-end formation.Glycosylation and sialylation of macrophage-derived human apolipoprotein E analyzed by SDS-PAGE and mass spectrometry: evidence for a novel site of glycosylation on Ser290.The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines.Carboxyl-terminal truncations alter the activity of the human α-galactosidase A A pharmacogenetic approach to identify mutant forms of α-galactosidase A that respond to a pharmacological chaperone for Fabry disease XBP1s Links the Unfolded Protein Response to the Molecular Architecture of Mature N-Glycans The Large Phenotypic Spectrum of Fabry Disease Requires Graduated Diagnosis and Personalized Therapy: A Meta-Analysis Can Help to Differentiate Missense Mutations Identification of a Caldariomyces fumago mutant secreting an inactive form of chloroperoxidase lacking the heme group and N-glycans.Structural and functional studies of the glycoside hydrolase family 3 β-glucosidase Cel3A from the moderately thermophilic fungus Rasamsonia emersonii.Effects of glycosylation on the stability of protein pharmaceuticals.N-(2-Aminoethyl) Ethanolamine-Induced Morphological, Biochemical, and Biophysical Alterations in Vascular Matrix Associated With Dissecting Aortic Aneurysm.Interrelationship of steric stabilization and self-crowding of a glycosylated protein.Role of glycosylation in conformational stability, activity, macromolecular interaction and immunogenicity of recombinant human factor VIII Structural aspects of therapeutic enzymes to treat metabolic disorders.Fabry disease, enzyme replacement therapy and the significance of antibody responses.Unglycosylation at Asn-633 made extracellular domain of E-cadherin folded incorrectly and arrested in endoplasmic reticulum, then sequentially degraded by ERAD.N-glycosylation is crucial for folding, trafficking, and stability of human tripeptidyl-peptidase I.Biosynthesis, glycosylation, and enzymatic processing in vivo of human tripeptidyl-peptidase I.Arabinosylation of recombinant human immunoglobulin-based protein therapeutics A novel bio-functional material based on mammalian cell aggresomes.Integrated approach to produce a recombinant, His-tagged human α-galactosidase A in mammalian cells.Endosomal lipid accumulation in NPC1 leads to inhibition of PKC, hypophosphorylation of vimentin and Rab9 entrapment.Protein signatures of molecular pathways in non-small cell lung carcinoma (NSCLC): comparison of glycoproteomics and global proteomics.Comparison of intracellular and secretion-based strategies for production of human α-galactosidase A in the filamentous fungus Trichoderma reesei.NPC1 late endosomes contain elevated levels of non-esterified ('free') fatty acids and an abnormally glycosylated form of the NPC2 protein.High overexpression of the human alpha-galactosidase A gene driven by its promoter in transgenic mice: implications for the treatment of Fabry disease.Roles of O-mannosylation of aberrant proteins in reduction of the load for endoplasmic reticulum chaperones in yeast.Characterization of a long-chain α-galactosidase from Papiliotrema flavescens.Substrate and Substrate-Mimetic Chaperone Binding Sites in Human α-Galactosidase A Revealed by Affinity-Mass Spectrometry.Strategies for the production of difficult-to-express full-length eukaryotic proteins using microbial cell factories: production of human alpha-galactosidase A.Phenotypic characteristics of the p.Asn215Ser (p.N215S) GLA mutation in male and female patients with Fabry disease: A multicenter Fabry Registry study.

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P2860

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

http://www.wikidata.org/entity/Q42196992

description

1998 nî lūn-bûn

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1998年の論文

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1998年学术文章

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1998年学术文章

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1998年学术文章

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1998年学术文章

@zh-my

1998年学术文章

@zh-sg

1998年學術文章

@yue

1998年學術文章

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1998年學術文章

@zh-hant

name

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@en

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@nl

type

label

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@en

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@nl

prefLabel

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@en

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@nl

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Biochemical Journal

P1476

Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

@en

P2093

K M Zeidner

http://www.w3.org/2001/XMLSchema#string

M E Grace

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R J Desnick

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Y A Ioannou

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P2860

Synthesis and processing of alpha-galactosidase A in human fibroblasts. Evidence for different mutations in Fabry disease

Affinity purification of alpha-galactosidase A from human spleen, placenta, and plasma with elimination of pyrogen contamination. Properties of the purified splenic enzyme compared to other forms

Molecular basis of Fabry disease: mutations and polymorphisms in the human alpha-galactosidase A gene

Overexpression of human alpha-galactosidase A results in its intracellular aggregation, crystallization in lysosomes, and selective secretion

Human alpha-galactosidase A: nucleotide sequence of a cDNA clone encoding the mature enzyme

Nucleotide sequence of the human alpha-galactosidase A gene

Cleavage of structural proteins during the assembly of the head of bacteriophage T4

Site-directed mutagenesis by overlap extension using the polymerase chain reaction

Conformational parameters for amino acids in helical, beta-sheet, and random coil regions calculated from proteins

An atypical variant of Fabry's disease with manifestations confined to the myocardium

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789-797

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scholarly article

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10.1042/BJ3320789

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332 ( Pt 3)

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1998-06-01T00:00:00Z

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13663383

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9620884

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1219542

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