Lysosomal acid alpha-glucosidase consists of four different peptides processed from a single chain precursor. - Wikidata - awgldk - TriplyDB

Lysosomal acid alpha-glucosidase consists of four different peptides processed from a single chain precursor.

Lysosomal acid alpha-glucosidase consists of four different peptides processed from a single chain precursor.

http://www.wikidata.org/entity/Q45133882

about

Cation-independent mannose 6-phosphate receptor: a composite of distinct phosphomannosyl binding sites Targeted approaches to induce immune tolerance for Pompe disease therapy Detection of Active Mammalian GH31 α-Glucosidases in Health and Disease Using In-Class, Broad-Spectrum Activity-Based Probes.Dysregulation of multiple facets of glycogen metabolism in a murine model of Pompe disease The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease N-glycans of recombinant human acid alpha-glucosidase expressed in the milk of transgenic rabbits.Pompe disease: from pathophysiology to therapy and back again Autophagy in skeletal muscle: implications for Pompe disease Molecular genetics of late onset glycogen storage disease II in Italy.Activity-based probes for functional interrogation of retaining β-glucuronidases.Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders.Pompe disease results in a Golgi-based glycosylation deficit in human induced pluripotent stem cell-derived cardiomyocytes.Predicting cross-reactive immunological material (CRIM) status in Pompe disease using GAA mutations: lessons learned from 10 years of clinical laboratory testing experience.Quantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann-Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis.Proteolytic processing of the neuronal ceroid lipofuscinosis related lysosomal protein CLN5.Clinical Laboratory Experience of Blood CRIM Testing in Infantile Pompe Disease Glycosylation-independent lysosomal targeting of acid α-glucosidase enhances muscle glycogen clearance in pompe mice.A multi-tiered analytical approach for the analysis and quantitation of high-molecular-weight aggregates in a recombinant therapeutic glycoprotein.Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease.Therapeutic approaches in glycogen storage disease type II/Pompe Disease.Biochemical and pharmacological characterization of different recombinant acid alpha-glucosidase preparations evaluated for the treatment of Pompe disease.The genotype-phenotype correlation in Pompe disease.α-Glucosidases and α-1,4-glucan lyases: structures, functions, and physiological actions.Antibody-mediated enzyme replacement therapy targeting both lysosomal and cytoplasmic glycogen in Pompe disease.The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblasts Biotherapeutic target or sink: analysis of the macrophage mannose receptor tissue distribution in murine models of lysosomal storage diseases.Splicing mutations in glycogen-storage disease type II: evaluation of the full spectrum of mutations and their relation to patients' phenotypes.A Portrait of the Human Organelle Proteome In Space and Time during Cytomegalovirus Infection Progress in Enzyme Replacement Therapy in Glycogen Storage Disease Type II Enzyme enhancers for the treatment of Fabry and Pompe disease.Structure of human lysosomal acid α-glucosidase-a guide for the treatment of Pompe disease.Abnormal mannose-6-phosphate receptor trafficking impairs recombinant alpha-glucosidase uptake in Pompe disease fibroblasts.Rescue of Pompe disease in mice by AAV-mediated liver delivery of secretable acid α-glucosidase.Structural modeling of mutant alpha-glucosidases resulting in a processing/transport defect in Pompe disease.Biochemical and structural study on a S529V mutant acid α-glucosidase responsive to pharmacological chaperones.

P2860

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P2860

Lysosomal acid alpha-glucosidase consists of four different peptides processed from a single chain precursor.

http://www.wikidata.org/entity/Q45133882

description

2004 nî lūn-bûn

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2004年の論文

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2004年学术文章

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2004年学术文章

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2004年学术文章

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2004年学术文章

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2004年学术文章

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2004年学术文章

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2004年學術文章

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2004年學術文章

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name

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

@en

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

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type

label

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

@en

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

@nl

prefLabel

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

@en

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

@nl

P1433

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P1433

Journal of Biological Chemistry

P1476

Lysosomal acid alpha-glucosida ...... from a single chain precursor.

@en

P2093

Karen L Albee

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Karen L Lee

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Kevin Brewer

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Robert D Cauthron

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Rodney J Moreland

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Roger W Decker

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Tim Edmunds

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William M Canfield

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X Kate Zhang

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Xiaoying Jin

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P2860

Computer assisted cloning of human neutral alpha-glucosidase C (GANC): a new paralog in the glycosyl hydrolase gene family 31.

Measurement of protein using bicinchoninic acid

Generalized glycogen storage and cardiomegaly in a knockout mouse model of Pompe disease

Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II

Activation of human acid sphingomyelinase through modification or deletion of C-terminal cysteine.

High-level production of recombinant human lysosomal acid alpha-glucosidase in Chinese hamster ovary cells which targets to heart muscle and corrects glycogen accumulation in fibroblasts from patients with Pompe disease

Characterization of glycoproteins and their associated oligosaccharides through the use of endoglycosidases.

Local alignment statistics.

The molecular heterogeneity of purified human liver lysosomal alpha-glucosidase (acid alpha-glucosidase).

Acid alpha-glucosidase: a new polymorphism in man demonstrable by 'affinity' electrophoresis.

P304

6780-6791

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P31

scholarly article

P356

10.1074/JBC.M404008200

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P407

P433

8

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P478

280

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P577

2004-11-01T00:00:00Z

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P698

15520017

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