(Q52037999)

20 April 2018

review article

1 reference

title

The GM2 activator protein, its roles as a co-factor in GM2 hydrolysis and as a general glycolipid transport protein. (English)

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20 April 2018

author name string

D J Mahuran

series ordinal

1

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20 April 2018

publication date

1 July 1998

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20 April 2018

Biochimica et Biophysica Acta

published in

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20 April 2018

volume

1393

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20 April 2018

issue

1

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20 April 2018

page(s)

1-18

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20 April 2018

AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2

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Identification of a processed pseudogene related to the functional gene encoding the GM2 activator protein: localization of the pseudogene to human chromosome 3 and the functional gene to human chromosome 5.

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Molecular Forms of GM2-Activator Protein. A Study on its Biosynthesis in Human Skin Fibroblasts

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A triple-binding-domain model explains the specificity of the interaction of a sphingolipid activator protein (SAP-1) with sulphatide, GM1-ganglioside and globotriaosylceramide

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The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein

7 January 2021

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7 January 2021

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Biochemical characterization of the Cys138Arg substitution associated with the AB variant form of GM2 gangliosidosis: evidence that Cys138 is required for the recognition of the GM2 activator/GM2 ganglioside complex by beta-hexosaminidase A

7 January 2021

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Biosynthesis, processing, and intracellular transport of GM2 activator protein in human epidermal keratinocytes. The lysosomal targeting of the GM2 activator is independent of a mannose-6-phosphate signal

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The mannose 6-phosphate receptor and the biogenesis of lysosomes

7 January 2021

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Selective recycling of the mannose 6-phosphate/IGF-II receptor to the trans Golgi network in vitro

7 January 2021

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Glucocerebrosidase, a lysosomal enzyme that does not undergo oligosaccharide phosphorylation

7 January 2021

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Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP )precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor

7 January 2021

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Over-expression of a functionally active human GM2-activator protein in Escherichia coli

7 January 2021

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Compartmental organization of the synthesis of GM3, GD3, and GM2 in golgi membranes from neural retina cells

7 January 2021

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Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases

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Glycosphingolipids in cellular interaction, differentiation, and oncogenesis

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Gangliosides are neuronal ligands for myelin-associated glycoprotein

7 January 2021

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The abnormal occurrence and the differentiation-dependent distribution of N-acetyl and N-glycolyl species of the ganglioside GM2 in human germ cell tumors. A study with specific monoclonal antibodies

7 January 2021

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Ganglioside modulation of the PDGF receptor. A model for ganglioside functions

7 January 2021

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Ganglioside GM1 binds to the Trk protein and regulates receptor function

7 January 2021

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N-Acetylcysteine-promoted Survival of PC12 Cells Is Glutathione-independent but Transcription-dependent

7 January 2021

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GM1 ganglioside activates the high-affinity nerve growth factor receptor trkA.

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7 January 2021

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7 January 2021

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Enzyme-linked Immunosorbent Assay for the Ganglioside GM2-Activator Protein. Screening of Normal Human Tissues and Body Fluids, of Tissues of GM2Gangliosidosis, and for Its Subcellular Localization

7 January 2021

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The protein activator specific for the enzymic hydrolysis of GM2 ganglioside in normal human brain and brains of three types of GM2 gangliosidosis

7 January 2021

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7 January 2021

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7 January 2021

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Characterization of the affinity of the G(M2) activator protein for glycolipids by a fluorescence dequenching assay

7 January 2021

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Complexing of Glycolipids and Their Transfer between Membranes by the Activator Protein for Degradation of Lysosomal Ganglioside GM2

7 January 2021

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Identification of a protein capable of causing fusion of endosome and lysosome membranes

7 January 2021

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The GM2 activator protein does not play a critical role in endosome and lysosome membrane fusion

7 January 2021

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Interaction of GM2 activator protein with glycosphingolipids

7 January 2021

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Binding and transport of gangliosides by prosaposin

7 January 2021

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7 January 2021

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Stimulation of a Ca(2+)-dependent protein kinase by GM1 ganglioside in nerve growth factor-treated PC12 cells

7 January 2021

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7 January 2021

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Activating Proteins for Ganglioside GM2Degradation by β-Hexosaminidase Isoenzymes in Tissue Extracts from Different Species

7 January 2021

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Specificity of mouse GM2 activator protein and beta-N-acetylhexosaminidases A and B. Similarities and differences with their human counterparts in the catabolism of GM2

7 January 2021

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Direct determination of the substrate specificity of the alpha-active site in heterodimeric beta-hexosaminidase A

7 January 2021

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Identification of functional domains within the alpha and beta subunits of beta-hexosaminidase A through the expression of alpha-beta fusion proteins

7 January 2021

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Formation of a ternary complex between GM2 activator protein, GM2 ganglioside and hexosaminidase A.

7 January 2021

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Specific recognition of N-acetylneuraminic acid in the GM2 epitope by human GM2 activator protein

7 January 2021

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Bacterial chitobiase structure provides insight into catalytic mechanism and the basis of Tay-Sachs disease

7 January 2021

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Hydrophobicity scales and computational techniques for detecting amphipathic structures in proteins

7 January 2021

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7 January 2021

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7 January 2021

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Characterization of an alternatively spliced GM2 activator protein, GM2A protein. An activator protein which stimulates the enzymatic hydrolysis of N-acetylneuraminic acid, but not N-acetylgalactosamine, from GM2.

7 January 2021

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The biochemistry of HEXA and HEXB gene mutations causing GM2 gangliosidosis

7 January 2021

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7 January 2021

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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

7 January 2021

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An unusual splicing mutation in the HEXB gene is associated with dramatically different phenotypes in patients from different racial backgrounds

7 January 2021

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Synthesis of 4-methylumbelliferyl-beta-D-N-acetylglucosamine-6-sulfate and its use in classification of GM2 gangliosidosis genotypes.

7 January 2021

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Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment

7 January 2021

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Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases

7 January 2021

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

7 January 2021

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Identification of a lysosomal protein causing lipid transfer, using a fluorescence assay designed to monitor membrane fusion between rat liver endosomes and lysosomes

7 January 2021

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7 January 2021

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Identifiers

DOI

10.1016/S0005-2760(98)00057-5

1 reference

20 April 2018

release_obthe5ve5rg7bdr5jm6wzvmceu

Fatcat ID

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https://api.fatcat.wiki/v0/release/obthe5ve5rg7bdr5jm6wzvmceu

mapped directly with Wikidata item

24 November 2022

based on heuristic

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20 April 2018