Journal ArticleDOI
Cleavage of the (1→3)-2-acetamido-2-deoxy-β-d-glucopyranosyl linkage present in keratan sulfate. The A and B isoenzymes of human liver hexosaminidase (EC 3.2.1.30)
Salvatore Toma,Giovanni Coppa,Patricia V. Donnelly,Roberta Ricci,Nicola Di Ferrante,Satish K. Srivastava +5 more
TLDR
The disaccharide 2-acetamido-2-deoxy-β- d -glucopyranosyl-(1→3)- d -[1-3H]-galactitol, prepared from keratan sulfate, was rapidly hydrolyzed by the A and B isoenzymes of normal human liver hexosaminidase (EC 3.2.1.30), and by the B isozyme prepared from the liver of a patient who had died of Tay-Sachs disease as discussed by the authors.About:
This article is published in Carbohydrate Research.The article was published on 1981-10-16. It has received 5 citations till now. The article focuses on the topics: Hexosaminidase & Sandhoff disease.read more
Citations
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Journal ArticleDOI
Heterogeneity of Morquio disease.
TL;DR: It is concluded that Morquio disease can be divided in several subgroups: besides the severe (“classical”) type A there exist an intermediate and a mild form that are also caused by a GalNAc‐6‐S sulfatase deficiency.
Journal ArticleDOI
Use of 3-O-(2-acetamido-3,4,6-tri-O-acetyl-2-deoxy-β-d-glucopyranosyl)-2,4,6-tri-O-acetyl-α-d-galactopyranosyl bromide as a glycosyl donor: Synthesis of p-nitrophenyl 3-O-(2-acetamido-2-deoxy-β-d-glucopyranosyl)-β-d-galactopyranoside
Saeed A. Abbas,Khushi L. Matta +1 more
TL;DR: In this article, the 3-O-(2-acetamido-3,4,6-tri-O-acetyl-2-deoxy-β-d -glucopyranosyl)-bromide was shown to be disaccharide.
Journal ArticleDOI
Synthesis of methyl 3-O- and 2-O-(2-acetamido-2-deoxy-β-d-glucopyranosyl)-α-d-galactopyranoside
Saeed A. Abbas,Khushi L. Matta +1 more
TL;DR: In this article, the structure of disaccharides was established by 1 H-n.m.n.r. spectroscopy and was confirmed by 13 Cn.
Journal ArticleDOI
Synthetic mucin fragments. Synthesis of a tetra- and two penta-saccharides containing the O-β-d-galactopyranosyl-(1→3)-O-(2-acetamido-2-deoxy-β-d-glucopyranosyl)-(1→3)-d-galactopyranose (“lacto-N-triose 1”) unit
Journal ArticleDOI
Chromatofocusing coupled with automated assay for beta-hexosaminidase isoenzymes in GM2 gangliosidosis.
TL;DR: The use of an automated and reliable method is described for the identification of homozygotes and carriers of the GM2 gangliosidosis.
References
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Journal Article
Protein Measurement with the Folin Phenol Reagent
TL;DR: Procedures are described for measuring protein in solution or after precipitation with acids or other agents, and for the determination of as little as 0.2 gamma of protein.
Journal ArticleDOI
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
TL;DR: This assay is very reproducible and rapid with the dye binding process virtually complete in approximately 2 min with good color stability for 1 hr with little or no interference from cations such as sodium or potassium nor from carbohydrates such as sucrose.
Journal ArticleDOI
N-Acetyl-β-glucosaminidases in human spleen
Donald Robinson,John L. Stirling +1 more
TL;DR: The N-acetyl-β-glucosaminidase of human spleen has been separated by gel electrophoresis into two components, an acidic form A and a basic form B, and evidence is presented to indicate that the A form contains a number of sialic acid residues.
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Anreicherung und Charakterisierung zweier Formen der menschlichen N-Acetyl-β-D-hexosaminidase
Konrad Sandhoff,Werner Wässle +1 more
Journal ArticleDOI
Spondyloepiphyseal dysplasia, corneal clouding, normal intelligence and acid β-galactosidase deficiency
TL;DR: A 14‐year‐old girl with a unique type of progressive spondyloepiphyseal dysplasia, corneal clouding, and no evidence of neurological abnormality, was found to have a remarkable deficiency of acid β‐galactosidase activity in cultured skin fibroblasts and in leucocyte preparations.