Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment
about
Animal models of GM2 gangliosidosis: utility and limitationsThe K-Cl cotransporter KCC3 is mutant in a severe peripheral neuropathy associated with agenesis of the corpus callosumMice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedureNdrg1-deficient mice exhibit a progressive demyelinating disorder of peripheral nervesSpecificity of mouse GM2 activator protein and beta-N-acetylhexosaminidases A and B. Similarities and differences with their human counterparts in the catabolism of GM2Multi-system disorders of glycosphingolipid and ganglioside metabolismIntracranial V. cholerae sialidase protects against excitotoxic neurodegenerationA single intramuscular injection of rAAV-mediated mutant erythropoietin protects against MPTP-induced parkinsonism.Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.The mousetrap: what we can learn when the mouse model does not mimic the human disease.In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 gangliosideGM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice.Biosynthesis and degradation of mammalian glycosphingolipids.Ganglioside biochemistryThe mutant Moonwalker TRPC3 channel links calcium signaling to lipid metabolism in the developing cerebellum.Effect of structural modifications of ganglioside GM2 on intra-molecular carbohydrate-to-carbohydrate interaction and enzymatic susceptibility.IKKalpha, IKKbeta, and NEMO/IKKgamma are each required for the NF-kappa B-mediated inflammatory response program.Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo.Kidney sulfatides in mouse models of inherited glycosphingolipid disorders: determination by nano-electrospray ionization tandem mass spectrometry.Simultaneous quantification of lyso-neutral glycosphingolipids and neutral glycosphingolipids by N-acetylation with [3H]acetic anhydride.Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation.Peanut agglutinin high phenotype of activated CD8+ T cells results from de novo synthesis of CD45 glycans.Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study.Sphingolipid metabolism. Sphingoid analogs, sphingolipid activator proteins, and the pathology of the cell.Targeted deletion of GD3 synthase protects against MPTP-induced neurodegeneration.Lack of PPCA expression only partially coincides with lysosomal storage in galactosialidosis mice: indirect evidence for spatial requirement of the catalytic rather than the protective function of PPCA.Battery of Behavioral Tests Assessing General Locomotion, Muscular Strength, and Coordination in Mice.Niemann–Pick Disease versus acid sphingomyelinase deficiency
P2860
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P2860
Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment
description
1997 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
1997 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
article publié dans les Procee ...... f the United States of America
@fr
artículu científicu espublizáu en 1997
@ast
im Juli 1997 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 1997/07/22)
@sk
vědecký článek publikovaný v roce 1997
@cs
wetenschappelijk artikel (gepubliceerd op 1997/07/22)
@nl
наукова стаття, опублікована в липні 1997
@uk
name
Mouse model of GM2 activator d ...... pathology and motor impairment
@ast
Mouse model of GM2 activator d ...... pathology and motor impairment
@en
Mouse model of GM2 activator d ...... pathology and motor impairment
@nl
type
label
Mouse model of GM2 activator d ...... pathology and motor impairment
@ast
Mouse model of GM2 activator d ...... pathology and motor impairment
@en
Mouse model of GM2 activator d ...... pathology and motor impairment
@nl
prefLabel
Mouse model of GM2 activator d ...... pathology and motor impairment
@ast
Mouse model of GM2 activator d ...... pathology and motor impairment
@en
Mouse model of GM2 activator d ...... pathology and motor impairment
@nl
P2093
P2860
P921
P3181
P356
P1476
Mouse model of GM2 activator d ...... pathology and motor impairment
@en
P2093
A. Grinberg
A. Hoffmann
H. Westphal
J. N. Crawley
K. M. Miller
K. Sandhoff
M. P. McDonald
R. L. Proia
P2860
P304
P3181
P356
10.1073/PNAS.94.15.8138
P407
P577
1997-07-22T00:00:00Z