Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis
about
Neurologic, gastric, and opthalmologic pathologies in a murine model of mucolipidosis type IVImpaired neural differentiation of induced pluripotent stem cells generated from a mouse model of Sandhoff diseaseAge-dependent effects of A53T alpha-synuclein on behavior and dopaminergic functionMouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairmentDeletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease miceA genetic model of substrate deprivation therapy for a glycosphingolipid storage disorderInvariant NKT cells reduce the immunosuppressive activity of influenza A virus-induced myeloid-derived suppressor cells in mice and humansInhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycinFcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model micePostanesthetic effects of isoflurane on behavioral phenotypes of adult male C57BL/6J miceA novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.Criteria for validating mouse models of psychiatric diseasesA novel instrumented multipeg running wheel system, Step-Wheel, for monitoring and controlling complex sequential stepping in miceTorsinA hypofunction causes abnormal twisting movements and sensorimotor circuit neurodegenerationPhysiological substrates for human lysosomal beta -hexosaminidase S.Mitochondrial morphogenesis, dendrite development, and synapse formation in cerebellum require both Bcl-w and the glutamate receptor delta2Caloric restriction shortens lifespan through an increase in lipid peroxidation, inflammation and apoptosis in the G93A mouse, an animal model of ALS.Abnormal differentiation of Sandhoff disease model mouse-derived multipotent stem cells toward a neural lineageNovel strains of mice deficient for the vesicular acetylcholine transporter: insights on transcriptional regulation and control of locomotor behavior.Inclusion body myositis-like phenotype induced by transgenic overexpression of beta APP in skeletal muscle.A mild impairment of mitochondrial electron transport has sex-specific effects on lifespan and aging in mice.Intracranial V. cholerae sialidase protects against excitotoxic neurodegenerationVitamin D3 deficiency differentially affects functional and disease outcomes in the G93A mouse model of amyotrophic lateral sclerosis.A single intramuscular injection of rAAV-mediated mutant erythropoietin protects against MPTP-induced parkinsonism.The mousetrap: what we can learn when the mouse model does not mimic the human disease.Vitamin D(3) at 50x AI attenuates the decline in paw grip endurance, but not disease outcomes, in the G93A mouse model of ALS, and is toxic in females.Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.Pulmonary inflammation is regulated by the levels of the vesicular acetylcholine transporter.Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammalsDeletion of GAD67 in dopamine receptor-1 expressing cells causes specific motor deficitsDevelopment of mammalian artificial chromosomes for the treatment of genetic diseases: Sandhoff and Krabbe diseases.MicroNeurotrophins Improve Survival in Motor Neuron-Astrocyte Co-Cultures but Do Not Improve Disease Phenotypes in a Mutant SOD1 Mouse Model of Amyotrophic Lateral Sclerosis.Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases.Quantitative measurement of postural sway in mouse models of human neurodegenerative diseaseProtease-resistant modified human β-hexosaminidase B ameliorates symptoms in GM2 gangliosidosis modelSphingosine kinase 1/S1P receptor signaling axis controls glial proliferation in mice with Sandhoff disease.Immunologic glycosphingolipidomics and NKT cell development in mouse thymus.Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.Enhancement of neuromuscular dynamics and strength behavior using extremely low magnitude mechanical signals in mice.
P2860
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P2860
Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis
description
1996 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
articolo scientifico
@it
artículu científicu espublizáu en 1996
@ast
im November 1996 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 1996/11/01)
@sk
vědecký článek publikovaný v roce 1996
@cs
wetenschappelijk artikel (gepubliceerd op 1996/11/01)
@nl
наукова стаття, опублікована в листопаді 1996
@uk
name
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@ast
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@en
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@nl
type
label
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@ast
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@en
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@nl
prefLabel
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@ast
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@en
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@nl
P2093
P3181
P356
P1433
P1476
Mice lacking both subunits of ...... osis and mucopolysaccharidosis
@en
P2093
A. Hoffmann
C. J. Tifft
C. M. Starr
J. N. Crawley
K. Sandhoff
M. L. Mack
M. P. McDonald
P2860
P2888
P304
P3181
P356
10.1038/NG1196-348
P407
P577
1996-11-01T00:00:00Z