scholarly journals Intravenous injection of l-BMAA induces a rat model with comprehensive characteristics of amyotrophic lateral sclerosis/Parkinson–dementia complex

2016 ◽  
Vol 5 (1) ◽  
pp. 79-96 ◽  
Author(s):  
Ke-Wei Tian ◽  
Hong Jiang ◽  
Bei-Bei Wang ◽  
Fan Zhang ◽  
Shu Han
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Amino Acid ◽  
Intravenous Injection ◽  
Rat Model ◽  
Protein Amino Acid ◽  
High Incidence ◽  
Parkinson Dementia ◽  
Lateral Sclerosis

Non-protein amino acid beta-N-methylamino-l-alanine (l-BMAA) is a neurotoxin that was associated with the high incidence of Amyotrophic Lateral Sclerosis/Parkinson–Dementia Complex (ALS/PDC) in Guam.

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

scholarly journals Questioning on the role of D amino acid oxidase in familial amyotrophic lateral sclerosis

2010 ◽  
Vol 107 (26) ◽  
pp. E107-E107 ◽  
Author(s):  
S. Millecamps ◽  
S. Da Barroca ◽  
C. Cazeneuve ◽  
F. Salachas ◽  
P.-F. Pradat ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Amino Acid ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Acid Oxidase ◽  
Amino Acid Oxidase ◽  
Lateral Sclerosis

Disappearance of high-incidence amyotrophic lateral sclerosis and parkinsonism-dementia on Guam

Neurology ◽  
1985 ◽  
Vol 35 (2) ◽  
pp. 193-193 ◽  
Author(s):  
R. M. Garruto ◽  
R. Yanagihara ◽  
D. C. Gajdusek
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
High Incidence ◽  
Lateral Sclerosis

scholarly journals Gonadectomy and dehydroepiandrosterone (DHEA) do not modulate disease progression in the G93A mutant SOD1 rat model of amyotrophic lateral sclerosis

2012 ◽  
Vol 13 (3) ◽  
pp. 311-314 ◽  
Author(s):  
Antonio Hayes-Punzo ◽  
Patrick Mulcrone ◽  
Michael Meyer ◽  
Jacalyn Mchugh ◽  
Clive N. Svendsen ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Rat Model ◽  
Mutant Sod1 ◽  
Lateral Sclerosis

Mesenchymal stromal cells prolong the lifespan in a rat model of amyotrophic lateral sclerosis

Cytotherapy ◽  
2011 ◽  
Vol 13 (9) ◽  
pp. 1036-1046 ◽  
Author(s):  
Serhiy Forostyak ◽  
Pavla Jendelova ◽  
Miroslava Kapcalova ◽  
David Arboleda ◽  
Eva Sykova
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mesenchymal Stromal Cells ◽  
Rat Model ◽  
Stromal Cells ◽  
Lateral Sclerosis
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