Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases
about
Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model.Impaired neural differentiation of induced pluripotent stem cells generated from a mouse model of Sandhoff diseaseLong-term correction of Sandhoff disease following intravenous delivery of rAAV9 to mouse neonatesMice doubly-deficient in lysosomal hexosaminidase A and neuraminidase 4 show epileptic crises and rapid neuronal lossNatural history of infantile G(M2) gangliosidosis.Pathology of GM2 gangliosidosis in Jacob sheepIminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff diseaseDeletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease miceA genetic model of substrate deprivation therapy for a glycosphingolipid storage disorderTay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradationFcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model miceIn Vivo NMR Studies of the Brain with Hereditary or Acquired Metabolic Disorders.Thymic alterations in GM2 gangliosidoses model mice.Prostaglandin E2 reverses aberrant production of an inflammatory chemokine by microglia from Sandhoff disease model mice through the cAMP-PKA pathwayMulti-system disorders of glycosphingolipid and ganglioside metabolismMesenchymal stem cells as cellular vectors for pediatric neurological disordersThe Caenorhabditis elegans mucolipin-like gene cup-5 is essential for viability and regulates lysosomes in multiple cell types.Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff diseaseFrom gene transfer to gene therapy in lysosomal storage diseases affecting the central nervous system.On the role of natural killer cells in neurodegenerative diseasesGlycosphingolipid lysosomal storage diseases: therapy and pathogenesis.Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses.Methionine sulfoxide reductase A (MsrA) protects cultured mouse embryonic stem cells from H2O2-mediated oxidative stress.Elevation of GM2 ganglioside during ethanol-induced apoptotic neurodegeneration in the developing mouse brain.Peripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.Development of mammalian artificial chromosomes for the treatment of genetic diseases: Sandhoff and Krabbe diseases.Storage solutions: treating lysosomal disorders of the brain.Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation.TSPO in a murine model of Sandhoff disease: presymptomatic marker of neurodegeneration and disease pathophysiology.AAV-mediated gene delivery in a feline model of Sandhoff disease corrects lysosomal storage in the central nervous system.Patterns of cell signaling pathway activation that characterize mammary development.Lysosomal Disorders Drive Susceptibility to Tuberculosis by Compromising Macrophage Migration.Anti-GM2 ganglioside antibodies are a biomarker for acute canine polyradiculoneuritis.Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation.Sequential proteolysis and high-field FTICR MS to determine disulfide connectivity and 4-maleimide TEMPO spin-label location in L126C GM2 activator proteinBone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.Reversibility of neuropathology in Tay-Sachs-related diseases.Lysosomal storage disease: revealing lysosomal function and physiology.Neuronopathic lysosomal storage diseases: clinical and pathologic findings.
P2860
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P2860
Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases
description
1997 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1997 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 1997
@ast
im Oktober 1997 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 1997/10/01)
@sk
vědecký článek publikovaný v roce 1997
@cs
wetenschappelijk artikel (gepubliceerd op 1997/10/01)
@nl
наукова стаття, опублікована в жовтні 1997
@uk
مقالة علمية (نشرت في أكتوبر 1997)
@ar
name
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@ast
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@en
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@nl
type
label
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@ast
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@en
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@nl
prefLabel
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@ast
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@en
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@nl
P2093
P921
P356
P1476
Apoptotic cell death in mouse ...... ay-Sachs and Sandhoff diseases
@en
P2093
J. M. Trasler
J. Michaud
J. Q. Huang
R. A. Gravel
S. Igdoura
P304
P356
10.1093/HMG/6.11.1879
P577
1997-10-01T00:00:00Z