Jefferson Weinberg ALS Center
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A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis.
The Journal of biological chemistry Nov, 2007 | Pubmed ID: 17823119
Nordihydroguaiaretic acid increases glutamate uptake in vitro and in vivo: therapeutic implications for amyotrophic lateral sclerosis.
Experimental neurology Sep, 2008 | Pubmed ID: 18625223
ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2.
Human molecular genetics Aug, 2010 | Pubmed ID: 20460269
Voltage-dependent inwardly rectifying potassium conductance in the outer membrane of neuronal mitochondria.
The Journal of biological chemistry Aug, 2010 | Pubmed ID: 20551319
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.
Nature neuroscience Nov, 2010 | Pubmed ID: 20953194
Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2.
Glia Nov, 2011 | Pubmed ID: 21769946
In vivo and in vitro determination of cell death markers in neurons.
Methods in molecular biology (Clifton, N.J.) , 2011 | Pubmed ID: 21913091
An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1.
Proceedings of the National Academy of Sciences of the United States of America Mar, 2012 | Pubmed ID: 22416121
Selective increase of two ABC drug efflux transporters at the blood-spinal cord barrier suggests induced pharmacoresistance in ALS.
Neurobiology of disease Aug, 2012 | Pubmed ID: 22521463
Small peptides against the mutant SOD1/Bcl-2 toxic mitochondrial complex restore mitochondrial function and cell viability in mutant SOD1-mediated ALS.
The Journal of neuroscience : the official journal of the Society for Neuroscience Jul, 2013 | Pubmed ID: 23843527
Defects in synapse structure and function precede motor neuron degeneration in Drosophila models of FUS-related ALS.
The Journal of neuroscience : the official journal of the Society for Neuroscience Dec, 2013 | Pubmed ID: 24336723
Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis.
Biochimica et biophysica acta Aug, 2014 | Pubmed ID: 24568860
ABC transporter-driven pharmacoresistance in Amyotrophic Lateral Sclerosis.
Brain research May, 2015 | Pubmed ID: 25175835
Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death.
Neuron , | Pubmed ID: 25521377
Inhibiting drug efflux transporters improves efficacy of ALS therapeutics.
Annals of clinical and translational neurology Dec, 2014 | Pubmed ID: 25574474
Regulation of ABC efflux transporters at blood-brain barrier in health and neurological disorders.
Brain research Dec, 2015 | Pubmed ID: 26187753
Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity.
Acta neuropathologica , | Pubmed ID: 26728149
Astrocytes drive upregulation of the multidrug resistance transporter ABCB1 (P-Glycoprotein) in endothelial cells of the blood-brain barrier in mutant superoxide dismutase 1-linked amyotrophic lateral sclerosis.
Glia 08, 2016 | Pubmed ID: 27158936
Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.
Neuroscience letters 01, 2017 | Pubmed ID: 27619540
Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72-ALS/FTD.
Cell reports 10, 2016 | Pubmed ID: 27732842
Mutation of the caspase-3 cleavage site in the astroglial glutamate transporter EAAT2 delays disease progression and extends lifespan in the SOD1-G93A mouse model of ALS.
Experimental neurology 06, 2017 | Pubmed ID: 28342750
Blood-Brain Barrier Driven Pharmacoresistance in Amyotrophic Lateral Sclerosis and Challenges for Effective Drug Therapies.
The AAPS journal 11, 2017 | Pubmed ID: 28779378
Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha.
Glia , | Pubmed ID: 29380416
Excess glutamate secreted from astrocytes drives upregulation of P-glycoprotein in endothelial cells in amyotrophic lateral sclerosis.
Experimental neurology 06, 2019 | Pubmed ID: 30974102
AAV2-BDNF promotes respiratory axon plasticity and recovery of diaphragm function following spinal cord injury.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 12, 2019 | Pubmed ID: 31577916
Synaptic dysfunction induced by glycine-alanine dipeptides in C9orf72-ALS/FTD is rescued by SV2 replenishment.
EMBO molecular medicine 05, 2020 | Pubmed ID: 32347002
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