Morphological differences between the atrophied small muscle fibres in amyotrophic lateral sclerosis and Werdnig-Hoffmann disease

1976 ◽  
Vol 34 (4) ◽  
pp. 321-327 ◽  
Author(s):  
Anna Fidziańska
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Muscle Fibres ◽  
Small Muscle ◽  
Morphological Differences ◽  
Lateral Sclerosis ◽  
Werdnig Hoffmann

scholarly journals Increased ROS-Dependent Fission of Mitochondria Causes Abnormal Morphology of the Cell Powerhouses in a Murine Model of Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 2021 ◽  
pp. 1-16
Author(s):  
Jan Stein ◽  
Bernd Walkenfort ◽  
Hilal Cihankaya ◽  
Mike Hasenberg ◽  
Verian Bader ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Mitochondrial Dynamics ◽  
Structured Illumination ◽  
Expression Studies ◽  
Morphological Differences ◽  
Als Patients ◽  
Insight Into ◽  
Lateral Sclerosis ◽  
Stable Stage

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease in humans and remains to have a fatal prognosis. Recent studies in animal models and human ALS patients indicate that increased reactive oxygen species (ROS) play an important role in the pathogenesis. Considering previous studies revealing the influence of ROS on mitochondrial physiology, our attention was focused on mitochondria in the murine ALS model, wobbler mouse. The aim of this study was to investigate morphological differences between wild-type and wobbler mitochondria with aid of superresolution structured illumination fluorescence microscopy, TEM, and TEM tomography. To get an insight into mitochondrial dynamics, expression studies of corresponding proteins were performed. Here, we found significantly smaller and degenerated mitochondria in wobbler motor neurons at a stable stage of the disease. Our data suggest a ROS-regulated, Ox-CaMKII-dependent Drp1 activation leading to disrupted fission-fusion balance, resulting in fragmented mitochondria. These changes are associated with numerous impairments, resulting in an overall self-reinforcing decline of motor neurons. In summary, our study provides common pathomechanisms with other ALS models and human ALS cases confirming mitochondria and related dysfunctions as a therapeutic target for the treatment of ALS.

Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis

2005 ◽  
Vol 15 (5) ◽  
pp. 377-388 ◽  
Author(s):  
Julie D. Atkin ◽  
Rachel L. Scott ◽  
Jan M. West ◽  
Elizabeth Lopes ◽  
Alvin K.J. Quah ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Muscle Fibres ◽  
Fast Twitch Muscle ◽  
Fast Twitch ◽  
Lateral Sclerosis

Intact single muscle fibres from SOD1 G93A amyotrophic lateral sclerosis mice display preserved specific force, fatigue resistance and training‐like adaptations

2019 ◽  
Vol 597 (12) ◽  
pp. 3133-3146 ◽  
Author(s):  
Arthur J. Cheng ◽  
Ilary Allodi ◽  
Thomas Chaillou ◽  
Maja Schlittler ◽  
Niklas Ivarsson ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Fatigue Resistance ◽  
Muscle Fibres ◽  
Single Muscle ◽  
Specific Force ◽  
And Training ◽  
Lateral Sclerosis

Automated Acoustic Analysis of Oral Diadochokinesis to Assess Bulbar Motor Involvement in Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 63 (1) ◽  
pp. 59-73 ◽  
Author(s):  
Panying Rong
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Acoustic Analysis ◽  
Speaking Rate ◽  
Disease Stage ◽  
Healthy Controls ◽  
Tongue Movement ◽  
Major Barrier ◽  
Syllable Repetition ◽  
Oral Diadochokinesis ◽  
Lateral Sclerosis

Purpose The purpose of this article was to validate a novel acoustic analysis of oral diadochokinesis (DDK) in assessing bulbar motor involvement in amyotrophic lateral sclerosis (ALS). Method An automated acoustic DDK analysis was developed, which filtered out the voice features and extracted the envelope of the acoustic waveform reflecting the temporal pattern of syllable repetitions during an oral DDK task (i.e., repetitions of /tɑ/ at the maximum rate on 1 breath). Cycle-to-cycle temporal variability (cTV) of envelope fluctuations and syllable repetition rate (sylRate) were derived from the envelope and validated against 2 kinematic measures, which are tongue movement jitter (movJitter) and alternating tongue movement rate (AMR) during the DDK task, in 16 individuals with bulbar ALS and 18 healthy controls. After the validation, cTV, sylRate, movJitter, and AMR, along with an established clinical speech measure, that is, speaking rate (SR), were compared in their ability to (a) differentiate individuals with ALS from healthy controls and (b) detect early-stage bulbar declines in ALS. Results cTV and sylRate were significantly correlated with movJitter and AMR, respectively, across individuals with ALS and healthy controls, confirming the validity of the acoustic DDK analysis in extracting the temporal DDK pattern. Among all the acoustic and kinematic DDK measures, cTV showed the highest diagnostic accuracy (i.e., 0.87) with 80% sensitivity and 94% specificity in differentiating individuals with ALS from healthy controls, which outperformed the SR measure. Moreover, cTV showed a large increase during the early disease stage, which preceded the decline of SR. Conclusions This study provided preliminary validation of a novel automated acoustic DDK analysis in extracting a useful measure, namely, cTV, for early detection of bulbar ALS. This analysis overcame a major barrier in the existing acoustic DDK analysis, which is continuous voicing between syllables that interferes with syllable structures. This approach has potential clinical applications as a novel bulbar assessment.

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