Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A (cerebroside sulfatase) activity by inhibitors of thiol proteinases.
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Arylsulfatase A pseudodeficiency: loss of a polyadenylylation signal and N-glycosylation siteDefective oligomerization of arylsulfatase a as a cause of its instability in lysosomes and metachromatic leukodystrophyMice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cellsHeterogeneity in late-onset metachromatic leukodystrophy. Effect of inhibitors of cysteine proteinases.Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): six unique arylsulfatase B gene alleles causing variable disease phenotypes.Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAsTrafficking of lysosomal enzymes in normal and disease states.Lysosomal acid phosphatase is transported to lysosomes via the cell surface.Rationale for the clinical use of immunotoxins: monoclonal antibodies conjugated to ribosome-inactivating proteins.Characterization of four arylsulfatase A missense mutations G86D, Y201C, D255H, and E312D causing metachromatic leukodystrophy.Accumulation of sialic acid in endocytic compartments interferes with the formation of mature lysosomes. Impaired proteolytic processing of cathepsin B in fibroblasts of patients with lysosomal sialic acid storage disease.Human lysosomal acid phosphatase is transported as a transmembrane protein to lysosomes in transfected baby hamster kidney cells.Targeting of a lysosomal membrane protein: a tyrosine-containing endocytosis signal in the cytoplasmic tail of lysosomal acid phosphatase is necessary and sufficient for targeting to lysosomes.Internalization of blocking antibodies against mannose-6-phosphate specific receptorsCathepsin D precursors in clathrin-coated organelles from human fibroblasts.Antibody to mannose 6-phosphate specific receptor induces receptor deficiency in human fibroblastsRestoration of arylsulphatase A activity in human-metachromatic-leucodystrophy fibroblasts via retroviral-vector-mediated gene transfer.Restoration of arylsulphatase B activity in human mucopolysaccharidosis-type-VI fibroblasts by retroviral-vector-mediated gene transfer.Mr 46,000 mannose 6-phosphate specific receptor: its role in targeting of lysosomal enzymes.Mannose 6-phosphate-specific receptor is a transmembrane protein with a C-terminal extension oriented towards the cytosolThe functional consequences of mis-sense mutations affecting an intra-molecular salt bridge in arylsulphatase A.Neutral endopeptidase-24.11 (enkephalinase). Biosynthesis and localization in human fibroblastsProteolytic processing and glycosylation of cathepsin B. The role of the primary structure of the latent precursor and of the carbohydrate moiety for cell-type-specific molecular forms of the enzyme.Leucocyte arylsulphatase A activity and subtypes of chronic schizophrenia.Molecular Forms of GM2-Activator Protein. A Study on its Biosynthesis in Human Skin Fibroblasts
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Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A (cerebroside sulfatase) activity by inhibitors of thiol proteinases.
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on October 1983
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Juvenile and adult metachromat ...... hibitors of thiol proteinases.
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Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A
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type
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Juvenile and adult metachromat ...... hibitors of thiol proteinases.
@en
Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A
@nl
prefLabel
Juvenile and adult metachromat ...... hibitors of thiol proteinases.
@en
Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A
@nl
P2093
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Juvenile and adult metachromat ...... hibitors of thiol proteinases.
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von Figura K
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P304
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10.1073/PNAS.80.19.6066
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P577
1983-10-01T00:00:00Z