Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation
about
Leaky splicing mutation in the acid maltase gene is associated with delayed onset of glycogenosis type IIGlycogen metabolism in humansDetection of Active Mammalian GH31 α-Glucosidases in Health and Disease Using In-Class, Broad-Spectrum Activity-Based Probes.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 diseaseUltrastructural analysis of development of myocardium in calreticulin-deficient miceHigh-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 diseasePompe disease: from pathophysiology to therapy and back againAutophagy in skeletal muscle: implications for Pompe diseaseHydrostatic isolated limb perfusion with adeno-associated virus vectors enhances correction of skeletal muscle in Pompe disease.The pharmacological chaperone AT2220 increases recombinant human acid α-glucosidase uptake and glycogen reduction in a mouse model of Pompe disease.A novel mechanism of lysosomal acid sphingomyelinase maturation: requirement for carboxyl-terminal proteolytic processing.Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders.Pompe disease: literature review and case seriesMistargeting of the lectin ERGIC-53 to the endoplasmic reticulum of HeLa cells impairs the secretion of a lysosomal enzyme.Neutralizing antibodies to therapeutic enzymes: considerations for testing, prevention and treatmentClinical Laboratory Experience of Blood CRIM Testing in Infantile Pompe DiseaseGlycosylation-independent lysosomal targeting of acid α-glucosidase enhances muscle glycogen clearance in pompe mice.Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease.Enhanced efficacy of an AAV vector encoding chimeric, highly secreted acid alpha-glucosidase in glycogen storage disease type II.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.Roles and regulation of secretory and lysosomal acid sphingomyelinase.The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblastsalpha-Glucosidase and N-acetylglucosamine-6-sulphatase are the major mannose-6-phosphate glycoproteins in human urine.Pharmacological enhancement of α-glucosidase by the allosteric chaperone N-acetylcysteine.Lysosomal alpha-glucosidase: cell-specific processing and altered maturation in HT-29 colon cancer cells.Lysosomal glycogen catabolismGlycogen Reduction in Myotubes of Late-Onset Pompe Disease Patients Using Antisense Technology.GAA hydrolyzes alpha(1;4) linkages in lysosomal glycogenGAA hydrolyzes alpha(1;6) linkages in lysosomal glycogenDefective GAA does not hydrolyze lysosomal glycogenGAA hydrolyzes lysosomal glycogen
P2860
Q24314311-173E2167-884F-4AD2-86B2-E86781A4DB8CQ26751151-EE0DC921-4E4D-48A1-9009-A59EB069BB77Q27300714-FCF64608-DB2B-4DF3-889D-48EA310D0732Q28540925-E9486395-708E-4180-9593-F7DC5B1F2440Q33263966-EC01308C-5B69-44F6-871D-7583DD5C045EQ33598299-53BE138C-6196-48D8-A921-B20652AF364AQ34054935-CEC1F921-8C9D-4F38-9934-530E5642DB61Q34170362-278A74D2-6B1E-47CA-9F7F-DB605FCCC642Q34332408-AA3B5E2F-B984-4863-A0AF-1353EC72E36FQ34345151-AF421A16-0758-41B1-B724-9A4F7DF6FBBFQ34536996-C1F1C9A1-552A-4545-B297-CC5A89434E34Q35007910-8629C925-1CA6-4580-A59C-866265F11636Q35029971-6E9BE5C8-25CF-4DFF-BF5C-8FD2A894097DQ36256238-6972EB98-5911-4B8C-9B21-CCAD0185F584Q36338254-904BBAD8-9ED1-4655-A92C-90D08C633EFFQ36361194-FAF30841-F53A-45B3-A471-54A2350CF76BQ36543643-F958BAEF-6FD7-477D-B157-79CD7305DA08Q37219555-33A2A521-6D0B-41A6-BAC9-3479A9554D2FQ37259816-EEF5D3BD-F4E4-4A34-8654-8A8834FACF8EQ37329535-DFBCB1CB-4E4C-420C-9F42-A47DDC415B2BQ37414571-757F95AA-F960-4582-A76D-BE7139746BD8Q37454661-0291AADF-BF6B-461C-8040-99F57DC6AED8Q39508157-3C24E896-47F9-458F-870C-F748FBA93231Q41833706-DA2938BF-8CD7-4AC2-B274-B15758CBCC17Q41912890-FF880C85-FD7A-4180-8B05-610E496FE43BQ42160250-4B1F817E-B6A6-4FA7-9B06-4E8B6C1E13BBQ45317169-1BE61DF3-5813-43DE-BD31-FE5FE86269FFQ48004397-6297EA2A-D95A-4B4C-B96F-A2A0FFBCF076Q50293122-7C06E1A7-9150-4686-BC0C-B093392A304BQ50293123-A6E6FCC9-F874-482F-A8C9-2DFA37718255Q55214598-8E800463-4098-4523-8EC5-16963448A8AFQ55216397-CB8A29C0-A23C-4ABE-ABAC-3DCF2BE60434
P2860
Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation
description
1993 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
1993 թվականի հունվարին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique Journal of Biological Chemistry
@fr
artículu científicu espublizáu en 1993
@ast
im Januar 1993 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 1993/01/25)
@sk
vědecký článek publikovaný v roce 1993
@cs
wetenschappelijk artikel (gepubliceerd op 1993/01/25)
@nl
наукова стаття, опублікована в січні 1993
@uk
name
Structural and functional chan ...... lular transport and maturation
@ast
Structural and functional chan ...... lular transport and maturation
@en
Structural and functional chan ...... lular transport and maturation
@nl
type
label
Structural and functional chan ...... lular transport and maturation
@ast
Structural and functional chan ...... lular transport and maturation
@en
Structural and functional chan ...... lular transport and maturation
@nl
prefLabel
Structural and functional chan ...... lular transport and maturation
@ast
Structural and functional chan ...... lular transport and maturation
@en
Structural and functional chan ...... lular transport and maturation
@nl
P2093
P3181
P1476
Structural and functional chan ...... lular transport and maturation
@en
P2093
Hermans MM
Wisselaar HA
van Beeumen J
P304
P3181
P407
P577
1993-01-01T00:00:00Z