The defect in Hurler and Hunter syndromes. II. Deficiency of specific factors involved in mucopolysaccharide degradation
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Less Is More: Substrate Reduction Therapy for Lysosomal Storage DisordersNDST1 Preferred Promoter Confirmation and Identification of Corresponding Transcriptional Inhibitors as Substrate Reduction Agents for Multiple Mucopolysaccharidosis DisordersThe defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidaseSimulation of genetic mucopolysaccharidoses in normal human fibroblasts by alteration of pH of the medium.Mannose 6 phosphorylation of lysosomal enzymes controls B cell functions.Validation of Urinary Glycosaminoglycans in Iranian patients with Mucopolysaccharidase type I: The effect of urine sedimentation characteristics.Hematopoietic cell transplantation for inherited metabolic diseases: an overview of outcomes and practice guidelines.The defect in the Hunter syndrome: deficiency of sulfoiduronate sulfatase.The genetic mismanagement of complex lipid metabolism.Somatic cell geneticsThe mucopolysaccharidoses (a review).The cornea--structure and macromolecules in health and disease. A reviewRegional assignment of the structural gene for human alpha-L-iduronidaseDelivery of lysosomal enzymes for therapeutic use: glucocerebrosidase as an example.Arylsulfatase A Overexpressing Human iPSC-derived Neural Cells Reduce CNS Sulfatide Storage in a Mouse Model of Metachromatic Leukodystrophy.Enzyme replacement therapy by fibroblast transplantation: long-term biochemical study in three cases of Hunter's syndrome.Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion.Implication of cord blood for cell-based therapy in refractory childhood diseases.Lysosomal enzyme replacement therapies: Historical development, clinical outcomes, and future perspectives.Combination Therapies for Lysosomal Storage Diseases: A Complex Answer to a Simple Problem.Long term survival and cardiopulmonary outcome in children with Hurler syndrome after haematopoietic stem cell transplantation.Detection of inborn errors of metabolism.Quantitative aspects of pinocytosis and the intracellular fate of N-acetyl-alpha-D-glucosaminidase in Sanfilippo B fibroblastsEndocytosis of sulphated proteoglycans by cultured skin fibroblasts.The disorder of hyaluronic acid metabolism in cultured skin fibroblasts derived from a patient with the Hurler syndrome.The excretion and degradation of chondroitin 4-sulphate administered to guinea pigs as free chondroitin sulphate and as proteoglycan.Regulation of heparan sulphate metabolism by adenosine 3':5'-cyclic monophosphate in hepatocytes in culture.Hurler's, Hunter's and Morquio's syndromes. A biochemical study in the light of current views of the underlying defects.Biochemical studies on the sulphated glycosaminoglycan fraction of skin fibroblasts cultured from a patient with the Hurler syndrome.Corrective factors for inborn errors of mucopolysaccharide metabolism.Lrp1/LDL Receptor Play Critical Roles in Mannose 6-Phosphate-Independent Lysosomal Enzyme Targeting.Changes in the incidence, patterns and outcomes of graft failure following hematopoietic stem cell transplantation for Hurler syndrome.
P2860
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P2860
The defect in Hurler and Hunter syndromes. II. Deficiency of specific factors involved in mucopolysaccharide degradation
description
1969 nî lūn-bûn
@nan
1969 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
1969 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
1969年の論文
@ja
1969年論文
@yue
1969年論文
@zh-hant
1969年論文
@zh-hk
1969年論文
@zh-mo
1969年論文
@zh-tw
1969年论文
@wuu
name
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@ast
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@en
type
label
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@ast
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@en
prefLabel
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@ast
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@en
P2093
P2860
P356
P1476
The defect in Hurler and Hunte ...... mucopolysaccharide degradation
@en
P2093
Fratantoni JC
Neufeld EF
P2860
P304
P356
10.1073/PNAS.64.1.360
P407
P577
1969-09-01T00:00:00Z