Mechanisms of disease: astrocytes in neurodegenerative disease

NJ Maragakis, JD Rothstein - Nature clinical practice Neurology, 2006 - nature.com
The term neurodegenerative disease refers to the principal pathology associated with
disorders such as amyotrophic lateral sclerosis, Alzheimer's disease, Huntington's disease and …

Glutamate transporters: animal models to neurologic disease

NJ Maragakis, JD Rothstein - Neurobiology of disease, 2004 - Elsevier
Glutamate is the primary excitatory amino acid neurotransmitter in the central nervous
system and its activity is carefully modulated in the synaptic cleft by glutamate transporters. A …

Glutamate transporters in neurologic disease

NJ Maragakis, JD Rothstein - Archives of neurology, 2001 - jamanetwork.com
The neurotoxic properties of glutamate were first demonstrated in 1957 by Lucas and
Newhouse, 1 who showed that systemic administration of glutamate to infant mice caused retinal …

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)

…, J Liu, Y She, B Goad, NJ Maragakis… - Proceedings of the …, 2002 - National Acad Sciences
Transgenic overexpression of Cu +2 /Zn +2 superoxide dismutase 1 (SOD1) harboring an
amyotrophic lateral sclerosis (ALS)-linked familial genetic mutation (SOD1 G93A ) in a …

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

…, G Periz, EAJ Simko, PG Shaw, MS Kim, NJ Maragakis… - Nature, 2014 - nature.com
A hexanucleotide repeat expansion (HRE), (GGGGCC) n , in C9orf72 is the most common
genetic cause of the neurodegenerative diseases amyotrophic lateral sclerosis (ALS) and …

Focal transplantation–based astrocyte replacement is neuroprotective in a model of motor neuron disease

…, AC Pardo, MS Rao, JD Rothstein, NJ Maragakis - Nature …, 2008 - nature.com
… We thank all members of the Maragakis and Rothstein labs for helpful discussion and K.
Tanaka for providing GLT1-null mice. This work was supported by the Robert Packard Center …

[HTML][HTML] Phase 1–2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS

…, S Ladha, AL Ludolph, NJ Maragakis… - … England Journal of …, 2020 - Mass Medical Soc
Background Tofersen is an antisense oligonucleotide that mediates the degradation of
superoxide dismutase 1 (SOD1) messenger RNA to reduce SOD1 protein synthesis. Intrathecal …

Human embryonic germ cell derivatives facilitate motor recovery of rats with diffuse motor neuron injury

…, J Lladó, MJ Shamblott, NJ Maragakis… - Journal of …, 2003 - Soc Neuroscience
We have investigated the potential of human pluripotent cells to restore function in rats
paralyzed with a virus-induced motor neuronopathy. Cells derived from embryonic germ cells, …

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo

…, AC Lepore, NJ Maragakis - Proceedings of the …, 2011 - National Acad Sciences
Recent studies highlight astrocytes as key drivers of motor neuron (MN) degeneration and
disease propagation in mutant human superoxide dismutase 1 (mSOD1)-mediated …

Neural stem cells protect against glutamate-induced excitotoxicity and promote survival of injured motor neurons through the secretion of neurotrophic factors

J Lladó, C Haenggeli, NJ Maragakis, EY Snyder… - Molecular and Cellular …, 2004 - Elsevier
Besides their capacity to give rise to neurons and/or glia, neural stem cells (NSCs) appear
to inherently secrete neurotrophic factors beneficial to injured neurons. To test this potential, …