The role of skeletal muscle in amyotrophic lateral sclerosis

JP Loeffler, G Picchiarelli, L Dupuis… - Brain …, 2016 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a fatal adult‐onset disease primarily characterized by
upper and lower motor neuron degeneration, muscle wasting and paralysis. It is increasingly …

[HTML][HTML] Skeletal muscle satellite cells, mitochondria, and microRNAs: their involvement in the pathogenesis of ALS

S Tsitkanou, PA Della Gatta, AP Russell - Frontiers in physiology, 2016 - frontiersin.org
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a fatal
motor neuron disorder. It results in progressive degeneration and death of upper and lower …

ALS‐linked protein disulfide isomerase variants cause motor dysfunction

U Woehlbier, A Colombo, MJ Saaranen, V Pérez… - The EMBO …, 2016 - embopress.org
Disturbance of endoplasmic reticulum (ER) proteostasis is a common feature of amyotrophic
lateral sclerosis (ALS). Protein disulfide isomerases (PDI s) are ER foldases identified as …

Strictly monitored exercise programs reduce motor deterioration in ALS: preliminary results of a randomized controlled trial

C Lunetta, A Lizio, VA Sansone, NM Cellotto… - Journal of …, 2016 - Springer
The objective of our study was to perform a randomized controlled trial (RCT) aimed to
evaluate the effects of three strictly monitored exercise programs (SMEP) compared to …

[HTML][HTML] Complement activation at the motor end-plates in amyotrophic lateral sclerosis

N Bahia El Idrissi, S Bosch, V Ramaglia… - Journal of …, 2016 - Springer
Background Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative
disease with no available therapy. Components of the innate immune system are activated …

AMPK signalling and defective energy metabolism in amyotrophic lateral sclerosis

ND Perera, BJ Turner - Neurochemical research, 2016 - Springer
Amyotrophic lateral sclerosis (ALS) is caused by selective loss of upper and lower motor
neurons by complex mechanisms that are incompletely understood. Motor neurons are …

[HTML][HTML] Synaptic failure: focus in an integrative view of ALS

C Casas, R Manzano, R Vaz, R Osta, D Brites - Brain plasticity, 2016 - content.iospress.com
From early description by Charcot, the classification of the Amyotrophic Lateral Sclerosis
(ALS) is evolving from a subtype of Motor Neuron (MN) Disease to be considered rather a …

[HTML][HTML] Unchanged neurotrophic factors and their receptors correlate with sparing in extraocular muscles in amyotrophic lateral sclerosis

VM Harandi, ARN Gaied, T Brännström… - … & visual science, 2016 - iovs.arvojournals.org
Purpose: To investigate the impact of amyotrophic lateral sclerosis (ALS) on the extraocular
muscles (EOMs) by examining the distribution of neurotrophic factors (NTFs) and their …

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

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 …

[HTML][HTML] Dynein disruption perturbs post-synaptic components and contributes to impaired MuSK clustering at the NMJ: implication in ALS

V Vilmont, B Cadot, E Vezin, F Le Grand, ER Gomes - Scientific Reports, 2016 - nature.com
The neuromuscular junction (NMJ) allows the transformation of a neuronal message into a
mechanical force by muscle contraction and is the target of several neuromuscular …