scholarly journals Functional skeletal muscle model derived from SOD1-mutant ALS patient iPSCs recapitulates hallmarks of disease progression

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Agnes Badu-Mensah ◽  
Xiufang Guo ◽  
Christopher W. McAleer ◽  
John W. Rumsey ◽  
James J. Hickman
Keyword(s):  
Skeletal Muscle ◽  
Disease Progression ◽  
Muscle Model ◽  
Sod1 Mutant

scholarly journals Autophagy in the heart is enhanced and independent of disease progression in mus musculus dystrophinopathy models

2019 ◽  
Vol 8 ◽  
pp. 204800401987958
Author(s):  
HR Spaulding ◽  
C Ballmann ◽  
JC Quindry ◽  
MB Hudson ◽  
JT Selsby
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Disease Progression ◽  
Gene Mutations ◽  
Dystrophin Gene ◽  
Mdx Mice ◽  
Dystrophin Deficiency ◽  
Muscle Wasting Disease ◽  
Dystrophin Protein

Background Duchenne muscular dystrophy is a muscle wasting disease caused by dystrophin gene mutations resulting in dysfunctional dystrophin protein. Autophagy, a proteolytic process, is impaired in dystrophic skeletal muscle though little is known about the effect of dystrophin deficiency on autophagy in cardiac muscle. We hypothesized that with disease progression autophagy would become increasingly dysfunctional based upon indirect autophagic markers. Methods Markers of autophagy were measured by western blot in 7-week-old and 17-month-old control (C57) and dystrophic (mdx) hearts. Results Counter to our hypothesis, markers of autophagy were similar between groups. Given these surprising results, two independent experiments were conducted using 14-month-old mdx mice or 10-month-old mdx/Utrn± mice, a more severe model of Duchenne muscular dystrophy. Data from these animals suggest increased autophagosome degradation. Conclusion Together these data suggest that autophagy is not impaired in the dystrophic myocardium as it is in dystrophic skeletal muscle and that disease progression and related injury is independent of autophagic dysfunction.

Tissue Engineered Skeletal Muscle Model of Rheumatoid Arthritis Using Human Primary Skeletal Muscle Cells

2021 ◽  
Author(s):  
Catherine E. Oliver ◽  
Hailee Patel ◽  
James Hong ◽  
Jonathan Carter ◽  
William E. Kraus ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Skeletal Muscle ◽  
Muscle Cells ◽  
Skeletal Muscle Cells ◽  
Muscle Model

scholarly journals An in vitro human skeletal muscle model: coculture of myotubes,neuron-like cells, and the capillary network

2017 ◽  
Vol 41 ◽  
pp. 514-525
Author(s):  
Ayşe Burcu ERTAN ◽  
Halime KENAR ◽  
Tahsin BEYZADEOĞLU ◽  
Fatma Neşe KÖK ◽  
Gamze TORUN KÖSE
Keyword(s):  
Skeletal Muscle ◽  
Human Skeletal Muscle ◽  
Capillary Network ◽  
Muscle Model

scholarly journals Potential therapeutic targets for ALS: MIR206, MIR208b and MIR499 are modulated during disease progression in the skeletal muscle of patients

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Lorena Di Pietro ◽  
Mirko Baranzini ◽  
Maria Grazia Berardinelli ◽  
Wanda Lattanzi ◽  
Mauro Monforte ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Disease Progression ◽  
Therapeutic Targets

scholarly journals Telomere length and age-dependent telomere attrition: the blood-and-muscle model

2019 ◽  
Vol 97 (4) ◽  
pp. 328-334 ◽  
Author(s):  
Mirna N. Chahine ◽  
Simon Toupance ◽  
Sandy El-Hakim ◽  
Carlos Labat ◽  
Sylvie Gautier ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Telomere Length ◽  
Cross Sectional Study ◽  
Muscle Model ◽  
Atherosclerotic Cardiovascular Disease ◽  
Cross Sectional ◽  
Telomere Attrition ◽  
Age Related ◽  
Age Dependent ◽  
Muscle Biopsies

Short telomere length (TL) is associated with atherosclerotic cardiovascular disease (ACVD) and other age-related diseases. It is unclear whether these associations originate from having inherently short TL or a faster TL attrition before or during disease development. We proposed the blood-and-muscle model to assess TL dynamics throughout life course. Our objective was to measure TL in leukocytes (LTL) and in skeletal muscle (MTL), which served as a proxy of TL at birth. The delta (MTL–LTL) represented life-long telomere attrition. Blood draws and skeletal muscle biopsies were performed on 35 Lebanese individuals undergoing surgery. Following DNA extraction, LTL and MTL were measured by Southern blot. In every individual aged between 30 and 85 years, MTL was longer than LTL. With age, MTL and LTL decreased, but the delta (MTL–LTL) increased by 14 bp/year. We validated the blood-and-muscle model that allowed us to identify TL, TL at birth, and lifelong TL attrition in a cross-sectional study. This model can be used in larger cross-sectional studies to evaluate the association of telomere dynamics with age-related diseases onset and progression.

scholarly journals ROS-related mitochondrial dysfunction in skeletal muscle of an ALS mouse model during the disease progression

2018 ◽  
Vol 138 ◽  
pp. 25-36 ◽  
Author(s):  
Yajuan Xiao ◽  
Chehade Karam ◽  
Jianxun Yi ◽  
Lin Zhang ◽  
Xuejun Li ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Mouse Model ◽  
Mitochondrial Dysfunction ◽  
Disease Progression

Treatment with lithium carbonate does not improve disease progression in two different strains of SOD1 mutant mice

2009 ◽  
Vol 10 (4) ◽  
pp. 221-228 ◽  
Author(s):  
Chiara Pizzasegola ◽  
Ilaria Caron ◽  
Cristina Daleno ◽  
Anna Ronchi ◽  
Claudio Minoia ◽  
...  
Keyword(s):  
Disease Progression ◽  
Lithium Carbonate ◽  
Mutant Mice ◽  
Different Strains ◽  
Sod1 Mutant

Simulation of upper limb motion by introducing continuum skeletal muscle model

2018 ◽  
Vol 2018.56 (0) ◽  
pp. 106
Author(s):  
Yuuta HUKUDA ◽  
Xian CHEN ◽  
Fei JIANG ◽  
Junji OHGI
Keyword(s):  
Skeletal Muscle ◽  
Upper Limb ◽  
Muscle Model
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