Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A
about
Evidence for two cDNA clones encoding human GM2-activator proteinOver-expression of a functionally active human GM2-activator protein in Escherichia coliVariant of GM2-gangliosidosis with hexosaminidase A having a severely changed substrate specificityAspartylglucosaminuria: cDNA encoding human aspartylglucosaminidase and the missense mutation causing the diseaseMolecular analysis of a GM2-activator deficiency in two patients with GM2-gangliosidosis AB variantPresence of activator proteins for the enzymic hydrolysis of GM1 and GM2 gangliosides in normal human urineGene transfer corrects acute GM2 gangliosidosis--potential therapeutic contribution of perivascular enzyme flowBiosynthesis and degradation of mammalian glycosphingolipids.GM2-ganglioside metabolism in hexosaminidase A deficiency states: determination in situ using labeled GM2 added to fibroblast cultures.Ganglioside GM2 N-acetyl-beta-D-galactosaminidase activity in cultured fibroblasts of late-infantile and adult GM2 gangliosidosis patients and of healthy probands with low hexosaminidase level.Ganglioside biochemistryDeciphering the role of CD1e protein in mycobacterial phosphatidyl-myo-inositol mannosides (PIM) processing for presentation by CD1b to T lymphocytes.Effect of structural modifications of ganglioside GM2 on intra-molecular carbohydrate-to-carbohydrate interaction and enzymatic susceptibility.Neuronal lysosomal enzyme replacement using fragment C of tetanus toxin.Ligand extraction properties of the GM2 activator protein and its interactions with lipid vesiclesThe immunological functions of saposins.My journey into the world of sphingolipids and sphingolipidoses.Advances on the Transfer of Lipids by Lipid Transfer ProteinsMitogenic effect of lysosomal hydrolases on bovine tracheal myocytes in culture.Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activityAdditional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase.Preliminary evidence for a processing error in the biosynthesis of Gaucher activator in mucolipidosis disease types II and III.Membrane-spanning lipids for an uncompromised monitoring of membrane fusion and intermembrane lipid transfer.Hexosaminidase A cleaves GalNAc from GM2 to form GM3
P2860
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P2860
Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A
description
1979 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1979 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 1979
@ast
scientific journal article
@en
vedecký článok (publikovaný 1979/12/01)
@sk
vědecký článek publikovaný v roce 1979
@cs
wetenschappelijk artikel (gepubliceerd op 1979/12/01)
@nl
наукова стаття, опублікована в грудні 1979
@uk
مقالة علمية (نشرت في ديسمبر 1979)
@ar
name
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@ast
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@en
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@nl
type
label
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@ast
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@en
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@nl
prefLabel
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@ast
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@en
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@nl
P1476
Purification and characterizat ...... M2 and GA2 by hexosaminidase A
@en
P2093
Conzelmann E
Sandhoff K
P304
P577
1979-12-01T00:00:00Z