Dysfunction of oligodendrocyte inwardly rectifying potassium channel in a rat model of amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Mina Peric ◽  
Ljiljana Nikolic ◽  
Pavle R. Andjus ◽  
Danijela Bataveljic
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Potassium Channel ◽  
Rat Model ◽  
Inwardly Rectifying Potassium Channel ◽  
Inwardly Rectifying ◽  
Lateral Sclerosis

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.

Intravenous infusion of mesenchymal stem cells delays disease progression in the SOD1G93A transgenic amyotrophic lateral sclerosis rat model

2021 ◽  
Vol 1757 ◽  
pp. 147296
Author(s):  
Hirotoshi Magota ◽  
Masanori Sasaki ◽  
Yuko Kataoka-Sasaki ◽  
Shinichi Oka ◽  
Ryo Ukai ◽  
...  
Keyword(s):  
Stem Cells ◽  
Amyotrophic Lateral Sclerosis ◽  
Mesenchymal Stem Cells ◽  
Disease Progression ◽  
Rat Model ◽  
Intravenous Infusion ◽  
Lateral Sclerosis

The inwardly rectifying potassium channel subunit Kir2.2v (KCNJN1) maps to 17p11.2→p11.1

1997 ◽  
Vol 79 (1-2) ◽  
pp. 85-87 ◽  
Author(s):  
N. Namba ◽  
R. Mori ◽  
H. Tanaka ◽  
I. Kondo ◽  
K. Narahara ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Inwardly Rectifying Potassium Channel ◽  
Inwardly Rectifying

scholarly journals NovelKCNJ10Gene Variations Compromise Function of Inwardly Rectifying Potassium Channel 4.1

2016 ◽  
Vol 291 (14) ◽  
pp. 7716-7726 ◽  
Author(s):  
Miguel P. Méndez-González ◽  
Yuriy V. Kucheryavykh ◽  
Astrid Zayas-Santiago ◽  
Wanda Vélez-Carrasco ◽  
Gerónimo Maldonado-Martínez ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Inwardly Rectifying Potassium Channel ◽  
Inwardly Rectifying
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