Appearance of phagocytic microglia adjacent to motoneurons in spinal cord tissue from a presymptomatic transgenic rat model of amyotrophic lateral sclerosis

2010 ◽  
pp. n/a-n/a ◽  
Author(s):  
Tomomi Sanagi ◽  
Shigeki Yuasa ◽  
Yasuko Nakamura ◽  
Eri Suzuki ◽  
Masashi Aoki ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Rat Model ◽  
Transgenic Rat ◽  
Spinal Cord Tissue ◽  
Lateral Sclerosis ◽  
Transgenic Rat Model

Riluzole Exhibits No Therapeutic Efficacy on a Transgenic Rat model of Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 17 (3) ◽  
pp. 275-285 ◽  
Author(s):  
Si Chen ◽  
Qiao Liao ◽  
Ke Lu ◽  
Jinxia Zhou ◽  
Cao Huang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Rat Model ◽  
Microglial Activation ◽  
Transgenic Rat ◽  
Intragastric Administration ◽  
Behavioral Deficits ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Transgenic Rat Model

Background: Amyotrophic lateral sclerosis (ALS) is a neurological disorder clinically characterized by motor system dysfunction, with intraneuronal accumulation of the TAR DNAbinding protein 43 (TDP-43) being a pathological hallmark. Riluzole is a primarily prescribed medicine for ALS patients, while its therapeutical efficacy appears limited. TDP-43 transgenic mice are existing animal models for mechanistic/translational research into ALS. Methods: We developed a transgenic rat model of ALS expressing a mutant human TDP-43 transgene (TDP-43M337V) and evaluated the therapeutic effect of Riluzole on this model. Relative to control, rats with TDP-43M337V expression promoted by the neurofilament heavy subunit (NEF) gene or specifically in motor neurons promoted by the choline acetyltransferase (ChAT) gene showed progressive worsening of mobility and grip strength, along with loss of motor neurons, microglial activation, and intraneuronal accumulation of TDP-43 and ubiquitin aggregations in the spinal cord. Results: Compared to vehicle control, intragastric administration of Riluzole (30 mg/kg/d) did not mitigate the behavioral deficits nor alter the neuropathologies in the transgenics. Conclusion: These findings indicate that transgenic rats recapitulate the basic neurological and neuropathological characteristics of human ALS, while Riluzole treatment can not halt the development of the behavioral and histopathological phenotypes in this new transgenic rodent model of ALS.

Toll-like receptor signaling in amyotrophic lateral sclerosis spinal cord tissue

Neuroscience ◽  
2011 ◽  
Vol 179 ◽  
pp. 233-243 ◽  
Author(s):  
M. Casula ◽  
A.M. Iyer ◽  
W.G.M. Spliet ◽  
J.J. Anink ◽  
K. Steentjes ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Spinal Cord Tissue ◽  
Receptor Signaling ◽  
Toll Like Receptor ◽  
Lateral Sclerosis

scholarly journals Retinoid receptors in chronic degeneration of the spinal cord: observations in a rat model of amyotrophic lateral sclerosis

2007 ◽  
Vol 103 (5) ◽  
pp. 1821-1833 ◽  
Author(s):  
Natasa Jokic ◽  
Yong Yong Ling ◽  
Rachael E. Ward ◽  
Adina T. Michael-Titus ◽  
John V. Priestley ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Rat Model ◽  
Retinoid Receptors ◽  
Lateral Sclerosis
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