Progressive impairment of CaV1.1 function in the skeletal muscle of mice expressing a mutant type 1 Cu/Zn superoxide dismutase (G93A) linked to amyotrophic …

D Beqollari, CF Romberg, G Dobrowolny, M Martini… - Skeletal muscle, 2016 - Springer
Background Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative
disorder that is typically fatal within 3–5 years of diagnosis. While motoneuron death is the …

Voltage-gated calcium channels are abnormal in cultured spinal motoneurons in the G93A-SOD1 transgenic mouse model of ALS

Q Chang, LJ Martin - Neurobiology of disease, 2016 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by
progressive loss of motoneurons. Hyperexcitability and excitotoxicity have been implicated …

Cu/Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of mitochondria and perturbs Ca2+ homeostasis in SOD1G93A …

MK Jaiswal, BU Keller - Molecular pharmacology, 2009 - ASPET
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by
the selective loss of defined motoneuron populations in the brainstem and spinal cord …

Hyperactive intracellular calcium signaling associated with localized mitochondrial defects in skeletal muscle of an animal model of amyotrophic lateral sclerosis

J Zhou, J Yi, R Fu, E Liu, T Siddique, E Ríos… - Journal of Biological …, 2010 - ASBMB
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disorder characterized by
degeneration of motor neurons and atrophy of skeletal muscle. Mutations in the superoxide …

Perturbations in intracellular Ca2+ handling in skeletal muscle in the G93A*SOD1 mouse model of amyotrophic lateral sclerosis

ER Chin, D Chen, KD Bobyk… - American Journal of …, 2014 - journals.physiology.org
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
characterized by skeletal muscle atrophy and weakness, ultimately leading to respiratory …

Intact single muscle fibres from SOD1G93A amyotrophic lateral sclerosis mice display preserved specific force, fatigue resistance and training‐like adaptations

AJ Cheng, I Allodi, T Chaillou, M Schlittler… - The Journal of …, 2019 - Wiley Online Library
Key points How defects in muscle contractile function contribute to weakness in amyotrophic
lateral sclerosis (ALS) were systematically investigated. Weakness in whole muscles from …

Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice

F von Lewinski, J Fuchs, BK Vanselow, BU Keller - Neuroscience letters, 2008 - Elsevier
Mutations in the Cu/Zn superoxide dismutase (SOD1) gene are associated with amyotrophic
lateral sclerosis (ALS), a fatal neurodegenerative disorder characterized by a selective …

Muscle contractility dysfunction precedes loss of motor unit connectivity in SOD1 (G93A) mice

CG Wier, AE Crum, AB Reynolds, CC Iyer… - Muscle & …, 2019 - Wiley Online Library
Introduction: Electrophysiological measurements are used in longitudinal clinical studies to
provide insight into the progression of amyotrophic lateral sclerosis (ALS) and the …

[HTML][HTML] Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 transgenic mouse model of amyotrophic lateral …

KHJ Park, I Vincent - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2008 - Elsevier
Amyotrophic lateral sclerosis (ALS) is primarily a motor neuron disorder. Intriguingly, early
muscle denervation preceding motor neuron loss is observed in mouse models of ALS …

Selective mitochondrial Ca2+ uptake deficit in disease endstage vulnerable motoneurons of the SOD1G93A mouse model of amyotrophic lateral sclerosis

A Fuchs, S Kutterer, T Mühling, J Duda… - The Journal of …, 2013 - Wiley Online Library
Key points• So far, increased excitability and calcium handling problems have been
discussed as causes for motoneuron death in amyotrophic lateral sclerosis (ALS) mainly on …