Physiological substrates for human lysosomal beta -hexosaminidase S. (Q31030090)

28 June 2017

title

Physiological substrates for human lysosomal beta -hexosaminidase S. (English)

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beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-ceramide(1-)

28 June 2017

main subject

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N-acetyl-beta-D-galactosaminyl-(1->4)-beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-ceramide(1-)

ChEBI release 2020-05-28

ChEBI ID

90163

1 reference

ChEBI release 2020-05-28

ChEBI ID

90164

author name string

Stefan T Hepbildikler

1

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28 June 2017

Roger Sandhoff

2

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28 June 2017

Melanie Kolzer

3

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28 June 2017

Richard L Proia

4

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28 June 2017

Konrad Sandhoff

5

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28 June 2017

language of work or name

English

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publication date

13 November 2001

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Journal of Biological Chemistry

28 June 2017

published in

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28 June 2017

volume

277

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28 June 2017

issue

4

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28 June 2017

page(s)

2562-2572

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Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease

28 June 2017

cites work

1 reference

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Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism

21 January 2018

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Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis

21 January 2018

1 reference

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Mice deficient in all forms of lysosomal beta-hexosaminidase show mucopolysaccharidosis-like pathology

21 January 2018

1 reference

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Synthesis of a human lysosomal enzyme, beta-hexosaminidase B, using the baculovirus expression system

21 January 2018

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Complete analysis of the glycosylation and disulfide bond pattern of human beta-hexosaminidase B by MALDI-MS.

21 January 2018

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Complete localization of disulfide bonds in GM2 activator protein

21 January 2018

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The Determination of Enzyme Dissociation Constants

21 January 2018

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Small-volume extrusion apparatus for preparation of large, unilamellar vesicles

21 January 2018

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Structure of heparan sulphate from human brain, with special regard to Alzheimer's disease

21 January 2018

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The use of polyacrylamide-gel electrophoresis for the high-resolution separation of reducing saccharides labelled with the fluorophore 8-aminonaphthalene-1,3,6-trisulphonic acid. Detection of picomolar quantities by an imaging system based on a cool

21 January 2018

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Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

21 January 2018

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Compositional analysis of glycosaminoglycans by electrospray mass spectrometry

21 January 2018

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Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase

21 January 2018

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Structure of Seven Oligosaccharides Excreted in the Urine of a Patient with Sandhoff's Disease (GM2 Gangliosidosis-Variant O)

21 January 2018

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A lipid associated with the antiphospholipid syndrome regulates endosome structure and function

21 January 2018

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Urinary excretion of sulphated N-acetylhexosamines in patients with various mucopolysaccharidoses

21 January 2018

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Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins

21 January 2018

1 reference

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Interfacial regulation of acid ceramidase activity. Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins

21 January 2018

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Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP.

21 January 2018

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Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators.

21 January 2018

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Degradation of membrane-bound ganglioside GM2 by beta -hexosaminidase A. Stimulation by GM2 activator protein and lysosomal lipids

21 January 2018

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Sulfolipids and glycolipid sulfotransferase activities in human renal cell carcinoma cells

21 January 2018

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Oligosaccharide ligands for NKR-P1 protein activate NK cells and cytotoxicity.

21 January 2018

1 reference

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The amino-terminal sequences in the pro-alpha and -beta polypeptides of human lysosomal beta-hexosaminidase A and B are retained in the mature isozymes

21 January 2018

1 reference

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W474C amino acid substitution affects early processing of the α-subunit of β-hexosaminidase A and is associated with subacute GM2 gangliosidosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105457200

Photoaffinity labeling of human lysosomal beta-hexosaminidase B. Identification of Glu-355 at the substrate binding site

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105457200

Serum immunoglobulins in Heymann's experimental nephritis modulate binding of properdin and factor-H to sulpho-glycosphingolipids II3SO3(-)-Gg3Cer and III3SO3(-)-,II3SO3(-)-Gg3Cer

21 January 2018

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Enzymic diagnosis of the genetic mucopolysaccharide storage disorders

21 January 2018

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Saccharide linkage analysis using methylation and other techniques

21 January 2018

1 reference

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21 January 2018

Identifiers

DOI

10.1074/JBC.M105457200

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28 June 2017

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