Storage solutions: treating lysosomal disorders of the brain. - Wikidata - wikimedia - TriplyDB

Storage solutions: treating lysosomal disorders of the brain.

Storage solutions: treating lysosomal disorders of the brain.

http://www.wikidata.org/entity/Q36210235

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The sensitivity of murine spermiogenesis to miglustat is a quantitative trait: a pharmacogenetic study Update on treatment of lysosomal storage diseases Murine muscle cell models for Pompe disease and their use in studying therapeutic approaches Lysosomal dysfunction promotes cleavage and neurotoxicity of tau in vivo Deletion in the N-terminal half of olfactomedin 1 modifies its interaction with synaptic proteins and causes brain dystrophy and abnormal behavior in mice.Cerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.Neuroprotection and lifespan extension in Ppt1(-/-) mice by NtBuHA: therapeutic implications for INCL Clues to neuro-degeneration in Niemann-Pick type C disease from global gene expression profiling Early changes in the apparent diffusion coefficient (ADC) in a mouse model of Sandhoff's disease occur prior to disease symptoms and behavioral deficits A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies.Lysosomal adaptation: how the lysosome responds to external cues The novel endosomal membrane protein Ema interacts with the class C Vps-HOPS complex to promote endosomal maturation.Nanomedicine in the diagnosis and therapy of neurodegenerative disorders.Transplantation and magnetic resonance imaging of canine neural progenitor cell grafts in the postnatal dog brain Bis(monoacylglycero)phosphate and ganglioside GM1 spontaneously form small homogeneous vesicles at specific concentrations.Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice Common and uncommon pathogenic cascades in lysosomal storage diseases Macroautophagy is not directly involved in the metabolism of amyloid precursor protein.The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis.Autophagy failure in Alzheimer's disease--locating the primary defect.Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain.Cell replacement therapy in neurological disease.Mammalian sialyltransferase ST3Gal-II: its exchange sialylation catalytic properties allow labeling of sialyl residues in mucin-type sialylated glycoproteins and specific gangliosides.Glia Disease and Repair-Remyelination The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol.Progenitor cell-based treatment of the pediatric myelin disorders Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C.Role of endolysosomes in HIV-1 Tat-induced neurotoxicity Astrocyte dysfunction triggers neurodegeneration in a lysosomal storage disorder.Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation.Lysosomal Disorders Drive Susceptibility to Tuberculosis by Compromising Macrophage Migration.Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy Development of a Rab9 transgenic mouse and its ability to increase the lifespan of a murine model of Niemann-Pick type C disease.Differential sensitivity of mouse strains to an N-alkylated imino sugar: glycosphingolipid metabolism and acrosome formation Structures of mammalian ER α-glucosidase II capture the binding modes of broad-spectrum iminosugar antivirals.Autophagy in neurodegeneration and development Differential regulation of sphingomyelin synthesis and catabolism in oligodendrocytes and neurons.Screening for pharmacological chaperones in Fabry disease Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy.A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

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P2860

Storage solutions: treating lysosomal disorders of the brain.

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Storage solutions: treating lysosomal disorders of the brain.

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Mylvaganam Jeyakumar

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Raymond A Dwek

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Terry D Butters

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P2860

Effect of non-steroidal anti-inflammatory drugs on risk of Alzheimer's disease: systematic review and meta-analysis of observational studies

NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin

A phase 1/2 clinical trial of enzyme replacement in fabry disease: pharmacokinetic, substrate clearance, and safety studies

Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin

Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice

Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases

A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder

Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism

Adenoviral gene therapy of the Tay-Sachs disease in hexosaminidase A-deficient knock-out mice

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