Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.
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Impaired neural differentiation of induced pluripotent stem cells generated from a mouse model of Sandhoff diseaseThe K-Cl cotransporter KCC3 is mutant in a severe peripheral neuropathy associated with agenesis of the corpus callosumIminosugar-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 disorderEffective gene therapy in an authentic model of Tay-Sachs-related diseasesSubstrate reduction therapy for glycosphingolipid storage disorders.Genes involved in the balance between neuronal survival and death during inflammationThymic alterations in GM2 gangliosidoses model mice.The glycosphingolipidoses-from disease to basic principles of metabolism.Prostaglandin E2 reverses aberrant production of an inflammatory chemokine by microglia from Sandhoff disease model mice through the cAMP-PKA pathwayIntracranial V. cholerae sialidase protects against excitotoxic neurodegenerationFrom gene transfer to gene therapy in lysosomal storage diseases affecting the central nervous system.A single intramuscular injection of rAAV-mediated mutant erythropoietin protects against MPTP-induced parkinsonism.Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses.Substrate reduction therapy in mouse models of the glycosphingolipidosesGanglioside biochemistryProduction of recombinant beta-hexosaminidase A, a potential enzyme for replacement therapy for Tay-Sachs and Sandhoff diseases, in the methylotrophic yeast Ogataea minutaPeripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation.AAV-mediated gene delivery in a feline model of Sandhoff disease corrects lysosomal storage in the central nervous system.Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff diseaseLysosomal Disorders Drive Susceptibility to Tuberculosis by Compromising Macrophage Migration.Peripheral blood mononuclear cell infiltration and neuroinflammation in the HexB-/- mouse model of neurodegeneration.Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice.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.Enhancement by Uridine Diphosphate of Macrophage Inflammatory Protein-1 Alpha Production in Microglia Derived from Sandhoff Disease Model Mice.Can the role of genetic factors in schizophrenia be enlightened by studies of candidate gene mutant mice behaviour?Suppression of the motor deficit in a mucolipidosis type IV mouse model by bone marrow transplantation.The cellular pathology of lysosomal diseases.Secretion of phosphomannosyl-deficient arylsulphatase A and cathepsin D from isolated human macrophagesDistribution and characterization of GFP(+) donor hematogenous cells in Twitcher mice after bone marrow transplantationWidespread correction of central nervous system disease after intracranial gene therapy in a feline model of Sandhoff disease.Proteolytic processing of the gamma-subunit is associated with the failure to form GlcNAc-1-phosphotransferase complexes and mannose 6-phosphate residues on lysosomal enzymes in human macrophages.Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease.Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy: effects on visceral and nervous system disease manifestations.Arginine supplementation improves rotorod performance in sickle transgenic mice.
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Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on May 1998
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@en
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@nl
type
label
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@en
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@nl
prefLabel
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@en
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@nl
P2093
P2860
P356
P1476
Bone marrow transplantation pr ...... ions in Sandhoff disease mice.
@en
P2093
A Hoffmann
G Goldstein
J N Crawley
K Sandhoff
M P McDonald
P2860
P304
P356
10.1172/JCI2127
P407
P577
1998-05-01T00:00:00Z