scholarly journals Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis (ALS)

10.3791/3936 ◽  
2012 ◽  
Author(s):  
René Günther ◽  
Martin Suhr ◽  
Jan C. Koch ◽  
Mathias Bähr ◽  
Paul Lingor ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Clinical Testing ◽  
Lateral Sclerosis

A longitudinal DTI and histological study of the spinal cord reveals early pathological alterations in G93A-SOD1 mouse model of amyotrophic lateral sclerosis

2017 ◽  
Vol 293 ◽  
pp. 43-52 ◽  
Author(s):  
Stefania Marcuzzo ◽  
Silvia Bonanno ◽  
Matteo Figini ◽  
Alessandro Scotti ◽  
Ileana Zucca ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Histological Study ◽  
G93a Sod1 Mouse ◽  
Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

scholarly journals Non-invasive diffusion tensor imaging detects white matter degeneration in the spinal cord of a mouse model of amyotrophic lateral sclerosis

NeuroImage ◽  
2011 ◽  
Vol 55 (2) ◽  
pp. 455-461 ◽  
Author(s):  
Clare K. Underwood ◽  
Nyoman D. Kurniawan ◽  
Tim J. Butler ◽  
Gary J. Cowin ◽  
Robyn H. Wallace
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Mouse Model ◽  
Diffusion Tensor ◽  
Non Invasive ◽  
White Matter Degeneration ◽  
Lateral Sclerosis

Impaired Pentose Phosphate Pathway in the Spinal Cord of the hSOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

2019 ◽  
Vol 56 (8) ◽  
pp. 5844-5855 ◽  
Author(s):  
Tesfaye Wolde Tefera ◽  
Katherine Bartlett ◽  
Shirley S. Tran ◽  
Mark P. Hodson ◽  
Karin Borges
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Pentose Phosphate Pathway ◽  
Pentose Phosphate ◽  
Lateral Sclerosis

Voltage-Dependent Sodium Channels in Spinal Cord Motor Neurons Display Rapid Recovery From Fast Inactivation in a Mouse Model of Amyotrophic Lateral Sclerosis

2006 ◽  
Vol 96 (6) ◽  
pp. 3314-3322 ◽  
Author(s):  
Cristina Zona ◽  
Massimo Pieri ◽  
Irene Carunchio
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Sodium Channels ◽  
Motor Neurons ◽  
Fast Inactivation ◽  
Patch Clamp Recording ◽  
Voltage Dependent ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a substantial loss of motor neurons in the spinal cord, brain stem, and motor cortex. Previous evidence showed that in a mouse model of a familial form of ALS expressing high levels of the human mutated protein Cu,Zn superoxide dismutase (Gly93→Ala, G93A), the firing properties of single motor neurons are altered to induce neuronal hyperexcitability. To determine whether the functionality of the macroscopic voltage-dependent Na+ currents is modified in G93A motor neurons, in the present work their physiological properties were examined. The voltage-dependent sodium channels were studied in dissociated motor neurons in culture from nontransgenic mice (Control), from transgenic mice expressing high levels of the human wild-type protein [superoxide dismutase 1 (SOD1)], and from G93A mice, using the whole cell configuration of the patch-clamp recording technique. The voltage dependency of activation and of steady-state inactivation, the kinetics of fast inactivation and slow inactivation of the voltage-dependent Na+ channels were not modified in the mutated mice. Conversely, the recovery from fast inactivation was significantly faster in G93A motor neurons than that in Control and SOD1. The recovery from fast inactivation was still significantly faster in G93A motor neurons exposed for different times (3–48 h) and concentrations (5–500 μM) to edaravone, a free-radical scavenger. Clarification of the importance of these changes in membrane ion channel functionality may have diagnostic and therapeutic implications in the pathogenesis of ALS.

scholarly journals Multicomponent diffusion analysis reveals microstructural alterations in spinal cord of a mouse model of amyotrophic lateral sclerosis ex vivo

PLoS ONE ◽  
2020 ◽  
Vol 15 (4) ◽  
pp. e0231598
Author(s):  
Jin Gao ◽  
Mingchen Jiang ◽  
Richard L. Magin ◽  
Rodolfo G. Gatto ◽  
Gerardo Morfini ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Ex Vivo ◽  
Multicomponent Diffusion ◽  
Microstructural Alterations ◽  
Diffusion Analysis ◽  
Lateral Sclerosis

scholarly journals A Cystine-Rich Whey Supplement (Immunocal®) Delays Disease Onset and Prevents Spinal Cord Glutathione Depletion in the hSOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

Antioxidants ◽  
2014 ◽  
Vol 3 (4) ◽  
pp. 843-865 ◽  
Author(s):  
Erika Ross ◽  
Aimee Winter ◽  
Heather Wilkins ◽  
Whitney Sumner ◽  
Nathan Duval ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Disease Onset ◽  
Glutathione Depletion ◽  
Lateral Sclerosis
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