scholarly journals MicroNeurotrophins Improve Survival in Motor Neuron-Astrocyte Co-Cultures but Do Not Improve Disease Phenotypes in a Mutant SOD1 Mouse Model of Amyotrophic Lateral Sclerosis

PLoS ONE ◽  
2016 ◽  
Vol 11 (10) ◽  
pp. e0164103 ◽  
Author(s):  
Kelly E. Glajch ◽  
Laura Ferraiuolo ◽  
Kaly A. Mueller ◽  
Matthew J. Stopford ◽  
Varsha Prabhkar ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Mutant Sod1 ◽  
Disease Phenotypes ◽  
Sod1 Mouse ◽  
Lateral Sclerosis

scholarly journals Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS

Metallomics ◽  
2016 ◽  
Vol 8 (9) ◽  
pp. 1002-1011 ◽  
Author(s):  
J. B. Hilton ◽  
A. R. White ◽  
P. J. Crouch
Keyword(s):  
Central Nervous System ◽  
Amyotrophic Lateral Sclerosis ◽  
Nervous System ◽  
Mouse Model ◽  
Limited Capacity ◽  
Mutant Sod1 ◽  
The Central Nervous System ◽  
Sod1 Mouse ◽  
Lateral Sclerosis

It is unclear why ubiquitous expression of mutant SOD1 selectively affects the central nervous system in amyotrophic lateral sclerosis. Here we hypothesise that the central nervous system is primarily affected because, unlike other tissues, it has relatively limited capacity to satiate an increased requirement for Cu.

Presymptomatic motor neuron loss and reactive astrocytosis in the SOD1 mouse model of amyotrophic lateral sclerosis

2001 ◽  
Vol 24 (11) ◽  
pp. 1510-1519 ◽  
Author(s):  
Sandra J. Feeney ◽  
Penelope A. McKelvie ◽  
Lawrence Austin ◽  
M.J. Bernadette Jean-Francois ◽  
Robert Kapsa ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Neuron Loss ◽  
Motor Neuron Loss ◽  
Sod1 Mouse ◽  
Lateral Sclerosis ◽  
Reactive Astrocytosis

scholarly journals Rilmenidine promotes MTOR-independent autophagy in the mutant SOD1 mouse model of amyotrophic lateral sclerosis without slowing disease progression

Autophagy ◽  
2017 ◽  
Vol 14 (3) ◽  
pp. 534-551 ◽  
Author(s):  
Nirma D. Perera ◽  
Rebecca K. Sheean ◽  
Chew L. Lau ◽  
Yea Seul Shin ◽  
Philip M. Beart ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Disease Progression ◽  
Mutant Sod1 ◽  
Sod1 Mouse ◽  
Lateral Sclerosis

Ascending neuropathology in the CNS of a mutant SOD1 mouse model of amyotrophic lateral sclerosis

2006 ◽  
Vol 1096 (1) ◽  
pp. 180-195 ◽  
Author(s):  
Anna Leichsenring ◽  
Bettina Linnartz ◽  
Xin-Ran Zhu ◽  
Hermann Lübbert ◽  
Christine C. Stichel
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Mutant Sod1 ◽  
Sod1 Mouse ◽  
Lateral Sclerosis

Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model

2009 ◽  
Vol 34 (3) ◽  
pp. 511-517 ◽  
Author(s):  
Bradley J. Turner ◽  
Nicholas J. Parkinson ◽  
Kay E. Davies ◽  
Kevin Talbot
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Survival Motor Neuron ◽  
Lateral Sclerosis

Potassium channel abnormalities are consistent with early axon degeneration of motor axons in the G127X SOD1 mouse model of amyotrophic lateral sclerosis

2017 ◽  
Vol 292 ◽  
pp. 154-167 ◽  
Author(s):  
Rikke Maglemose ◽  
Anne Hedegaard ◽  
Janna Lehnhoff ◽  
Kristina Petrova Dimintiyanova ◽  
Mihai Moldovan ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Potassium Channel ◽  
Axon Degeneration ◽  
Motor Axons ◽  
Sod1 Mouse ◽  
Lateral Sclerosis
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