A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
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Autophagy in lysosomal storage disordersDemonstration of the heterozygous state for I-cell disease and pseudo-Hurler polydystrophy by assay of N-acetylglucosaminylphosphotransferase in white blood cells and fibroblastsPhosphohexosyl recognition is a general characteristic of pinocytosis of lysosomal glycosidases by human fibroblastsImpaired microtubule function correctable by cyclic GMP and cholinergic agonists in the Chediak-Higashi syndromeSaccharide determinants in selective drug delivery.Sustained normalization of neurological disease after intracranial gene therapy in a feline model.The mucopolysaccharidoses.Replacement therapy in the mucopolysaccharidosesLC3A-positive light microscopy detected patterns of autophagy and prognosis in operable breast carcinomas.Mucolipidosis II and III. The genetic relationships between two disorders of lysosomal enzyme biosynthesis.Cellular uptake of saposin (SAP) precursor and lysosomal delivery by the low density lipoprotein receptor-related protein (LRP).Sialidosis: a review of human neuraminidase deficiency.A role for clathrin in the sorting of vacuolar proteins in the Golgi complex of yeastMolecular basis of variant pseudo-hurler polydystrophy (mucolipidosis IIIC)Enzymatic identification of mannose 6-phosphate on the recognition marker for receptor-mediated pinocytosis of beta-glucuronidase by human fibroblasts.A novel mouse model of a patient mucolipidosis II mutation recapitulates disease pathologyMucopolysaccharidoses and mucolipidoses.Enveloped virus acquires membrane defect when passaged in fibroblasts from I-cell disease patients.Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts.Evidence for specific recognition sites mediating clearance of lysosomal enzymes in vivoThe defect in the Hunter syndrome: deficiency of sulfoiduronate sulfatase.The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies.Apparently normal extracellular acidic alpha-mannosidase in fibroblast cultures from patients with mannosidosis.Electrophoretic abnormalities of lysosomal enzymes in mucolipidosis fibroblast linesMutant defective in processing of an enzyme located in the lysosome-like vacuole of Saccharomyces cerevisiae.Phenotypic diversity of human diseases resulting from allelic series.Characterization of beta-D-galactosidase isolated from I-cell disease liver.The mucopolysaccharidoses (a review).Fibroblast receptor for lysosomal enzymes mediates pinocytosis of multivalent phosphomannan fragment.Carbamycholine prevents giant granule-formation in cultured fibroblasts from beige (Chediak-Higashi) mice.Studies on cell adhesion and recognition. III. The occurrence of alpha-mannosidase at the fibroblast cell surface, and its possible role in cell recognition.Studies on cell adhesion and recognition. I. Extent and specificity of cell adhesion triggered by carbohydrate-reactive proteins (glycosidases and lectins) and by fibronectin.Studies of the biosynthesis of the mannose 6-phosphate receptor in receptor-positive and -deficient cell lines.Fate of secretory proteins trapped in oocytes of Xenopus laevis by disruption of the cytoskeleton or by imbalanced subunit synthesis.Phosphorylation, glycosylation, and proteolytic activity of the 52-kD estrogen-induced protein secreted by MCF7 cells.Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice.A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth.Redistribution of mannose-6-phosphate receptors induced by tunicamycin and chloroquine.Lysosomal Storage Diseases-Regulating Neurodegeneration.Dual pathways for ribonucleic acid turnover in WI-38 but not in I-cell human diploid fibroblasts.
P2860
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P2860
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
description
1972 nî lūn-bûn
@nan
1972年の論文
@ja
1972年論文
@yue
1972年論文
@zh-hant
1972年論文
@zh-hk
1972年論文
@zh-mo
1972年論文
@zh-tw
1972年论文
@wuu
1972年论文
@zh
1972年论文
@zh-cn
name
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
@en
type
label
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
@en
prefLabel
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
@en
P1476
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
@en
P2093
P304
P356
10.1016/0006-291X(72)90310-5
P577
1972-11-01T00:00:00Z