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 Spinal motoneurones are intrinsically more responsive in the adult G93A SOD1 mouse model of amyotrophic lateral sclerosis

2020 ◽  
Vol 598 (19) ◽  
pp. 4385-4403
Author(s):  
Dennis B. Jensen ◽  
Marion Kadlecova ◽  
Ilary Allodi ◽  
Claire F. Meehan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
G93a Sod1 Mouse ◽  
Spinal Motoneurones ◽  
Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

On the Relation of Oxidative Stress to Neuroinflammation: Lessons Learned from the G93A-SOD1 Mouse Model of Amyotrophic Lateral Sclerosis

2006 ◽  
Vol 8 (11-12) ◽  
pp. 2075-2087 ◽  
Author(s):  
Kenneth Hensley ◽  
Molina Mhatre ◽  
Shenyun Mou ◽  
Quentin N. Pye ◽  
Charles Stewart ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Lessons Learned ◽  
G93a Sod1 Mouse ◽  
Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotrophic lateral sclerosis

2007 ◽  
Vol 25 (6) ◽  
pp. 1669-1677 ◽  
Author(s):  
Heiko G. Niessen ◽  
Grazyna Debska-Vielhaber ◽  
Kerstin Sander ◽  
Frank Angenstein ◽  
Albert C. Ludolph ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Progression Markers ◽  
G93a Sod1 Mouse ◽  
Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

scholarly journals Evolution of the neurochemical profiles in the G93A-SOD1 mouse model of amyotrophic lateral sclerosis

2018 ◽  
Vol 39 (7) ◽  
pp. 1283-1298 ◽  
Author(s):  
Hongxia Lei ◽  
Elisabeth Dirren ◽  
Carole Poitry-Yamate ◽  
Bernard L Schneider ◽  
Rolf Gruetter ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Cortex ◽  
Mouse Model ◽  
Disease Progression ◽  
Resonance Spectroscopy ◽  
Neurochemical Profiles ◽  
G93a Sod1 Mouse ◽  
Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

In vivo 1H magnetic resonance spectroscopy (1H-MRS) investigations of amyotrophic lateral sclerosis (ALS) mouse brain may provide neurochemical profiles and alterations in association with ALS disease progression. We aimed to longitudinally follow neurochemical evolutions of striatum, brainstem and motor cortex of mice transgenic for G93A mutant human superoxide dismutase type-1 (G93A-SOD1), an ALS model. Region-specific neurochemical alterations were detected in asymptomatic G93A-SOD1 mice, particularly in lactate (−19%) and glutamate (+8%) of brainstem, along with γ-amino-butyric acid (−30%), N-acetyl-aspartate (−5%) and ascorbate (+51%) of motor cortex. With disease progression towards the end-stage, increased numbers of metabolic changes of G93A-SOD1 mice were observed (e.g. glutamine levels increased in the brainstem (>+66%) and motor cortex (>+54%)). Through ALS disease progression, an overall increase of glutamine/glutamate in G93A-SOD1 mice was observed in the striatum ( p < 0.01) and even more so in two motor neuron enriched regions, the brainstem and motor cortex ( p < 0.0001). These 1H-MRS data underscore a pattern of neurochemical alterations that are specific to brain regions and to disease stages of the G93A-SOD1 mouse model. These neurochemical changes may contribute to early diagnosis and disease monitoring in ALS patients.

Proteomic analysis of spinal cord of presymptomatic amyotrophic lateral sclerosis G93A SOD1 mouse

2007 ◽  
Vol 353 (3) ◽  
pp. 719-725 ◽  
Author(s):  
Tania Massignan ◽  
Filippo Casoni ◽  
Manuela Basso ◽  
Paola Stefanazzi ◽  
Emiliano Biasini ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Proteomic Analysis ◽  
G93a Sod1 Mouse ◽  
Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

Multiple transplants of hNT cells into the spinal cord of SOD1 mouse model of familial amyotrophic lateral sclerosis

2006 ◽  
Vol 7 (4) ◽  
pp. 227-232 ◽  
Author(s):  
Svitlana Garbuzova‐Davis ◽  
Alison E. Willing ◽  
Samuel Saporta ◽  
Eleanor B. Justen ◽  
Iwona E. Misiuta ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Sod1 Mouse ◽  
Lateral Sclerosis
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