scholarly journals A Two Stage Model of Skeletal Muscle Necrosis in Muscular Dystrophy - The Role of Fiber Branching in the Terminal Stage

10.5772/31880 ◽  
2012 ◽  
Author(s):  
Stewart Head
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Stage Model ◽  
Two Stage ◽  
Terminal Stage ◽  
Muscle Necrosis

TRANSAMINATION IN MUSCULAR DYSTROPHY AND THE EFFECT OF EXOGENOUS GLUTAMATE: A STUDY ON VITAMIN E DEFICIENT RABBITS, AND MICE WITH HEREDITARY DYSTROPHY

1963 ◽  
Vol 41 (1) ◽  
pp. 1423-1432 ◽  
Author(s):  
Roland O. Laferté ◽  
Harris Rosenkrantz ◽  
Louis Berlinguet
Keyword(s):  
Body Weight ◽  
Vitamin E ◽  
Muscular Dystrophy ◽  
Glutamic Acid ◽  
Alanine Transaminase ◽  
Deficient Diet ◽  
Terminal Stage ◽  
Weight Losses

A study of transaminase enzymes in various tissues of different species was carried out. Rabbits were fed with a vitamin E deficient diet. Controls receiving vitamin E were maintained on the same diet. Animals were killed at intervals and the glutamic-aspartic transaminase (GOT) and the glutamic-alanine transaminase (GPT) levels were determined in the muscle, blood, and liver.Mice with hereditary muscular dystrophy and normal litter mates which served as controls were killed at different stages of the disease and GPT and GOT levels were also determined in the muscle, blood, and liver.Important variations between the two types of dystrophy were noticed. Variations in the levels of GPT and GOT were also significant in blood and liver of dystrophic rabbits.Exogenous glutamic acid was injected to vitamin E deprived rabbits. Body weight losses and the onset of the terminal stage of the disease were much postponed when compared to the vitamin E deprived rabbits which did not receive glutamic acid.A discussion of the possible role of glutamic acid in muscular dystrophy of vitamin E deprived rabbits is presented.

scholarly journals Skeletal muscle secretome in Duchenne muscular dystrophy: a pivotal anti-inflammatory role of adiponectin

2017 ◽  
Vol 74 (13) ◽  
pp. 2487-2501 ◽  
Author(s):  
S. Lecompte ◽  
M. Abou-Samra ◽  
R. Boursereau ◽  
L. Noel ◽  
S. M. Brichard
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Anti Inflammatory

HAEMODYNAMIC CONTRIBUTIONS TO THE PATHOGENESIS OF PREECLAMPSIA AND ECLAMPSIA

2008 ◽  
Vol 19 (1) ◽  
pp. 85-104 ◽  
Author(s):  
IRA M BERNSTEIN ◽  
MARILYN J CIPOLLA
Keyword(s):  
Endothelial Injury ◽  
Trophoblast Invasion ◽  
Stage Model ◽  
Abnormal Placentation ◽  
Two Stage ◽  
Second Stage

Current hypotheses regarding the origins of preeclampsia have focused on the “Two stage model”. This model suggests that the primary steps in the pathophysiologic sequence of preeclampsia are initiated by abnormal placentation including the classic finding of abnormal trophoblast invasion of maternal decidual spiral arteries. The second stage of the sequence includes the elaboration of a single or multiple substances from these disordered placentas which contribute to the generalized maternal systemic illness, eventually manifesting as endothelial injury, hypertension and proteinuria. Recent studies have focused on the role of pro and anti-angiogenic peptides as potential placentally derived aetiologic agents in this pathophysiologic sequence, although other placental products have been highlighted in recent research. Despite the fact that this modeling of preeclampsia has widespread support significant limitations to this hypothesis can be identified.

scholarly journals Role of dystroglycan in limiting contraction-induced injury to the sarcomeric cytoskeleton of mature skeletal muscle

2016 ◽  
Vol 113 (39) ◽  
pp. 10992-10997 ◽  
Author(s):  
Erik P. Rader ◽  
Rolf Turk ◽  
Tobias Willer ◽  
Daniel Beltrán ◽  
Kei-ichiro Inamori ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Pathological Feature ◽  
Passive Tension ◽  
Lengthening Contractions ◽  
Mechanistic Insight ◽  
The Stability ◽  
Muscle Biopsies ◽  
Insight Into

Dystroglycan (DG) is a highly expressed extracellular matrix receptor that is linked to the cytoskeleton in skeletal muscle. DG is critical for the function of skeletal muscle, and muscle with primary defects in the expression and/or function of DG throughout development has many pathological features and a severe muscular dystrophy phenotype. In addition, reduction in DG at the sarcolemma is a common feature in muscle biopsies from patients with various types of muscular dystrophy. However, the consequence of disrupting DG in mature muscle is not known. Here, we investigated muscles of transgenic mice several months after genetic knockdown of DG at maturity. In our study, an increase in susceptibility to contraction-induced injury was the first pathological feature observed after the levels of DG at the sarcolemma were reduced. The contraction-induced injury was not accompanied by increased necrosis, excitation–contraction uncoupling, or fragility of the sarcolemma. Rather, disruption of the sarcomeric cytoskeleton was evident as reduced passive tension and decreased titin immunostaining. These results reveal a role for DG in maintaining the stability of the sarcomeric cytoskeleton during contraction and provide mechanistic insight into the cause of the reduction in strength that occurs in muscular dystrophy after lengthening contractions.

scholarly journals From Innate to Adaptive Immune Response in Muscular Dystrophies and Skeletal Muscle Regeneration: The Role of Lymphocytes

2014 ◽  
Vol 2014 ◽  
pp. 1-12 ◽  
Author(s):  
Luca Madaro ◽  
Marina Bouché
Keyword(s):  
Skeletal Muscle ◽  
Immune Response ◽  
Immune Cells ◽  
Current Knowledge ◽  
Adaptive Immune Response ◽  
Inflammatory Cells ◽  
Skeletal Muscle Regeneration ◽  
Current View ◽  
Muscle Necrosis

Skeletal muscle is able to restore contractile functionality after injury thanks to its ability to regenerate. Following muscle necrosis, debris is removed by macrophages, and muscle satellite cells (MuSCs), the muscle stem cells, are activated and subsequently proliferate, migrate, and form muscle fibers restoring muscle functionality. In most muscle dystrophies (MDs), MuSCs fail to properly proliferate, differentiate, or replenish the stem cell compartment, leading to fibrotic deposition. However, besides MuSCs, interstitial nonmyogenic cells and inflammatory cells also play a key role in orchestrating muscle repair. A complete understanding of the complexity of these mechanisms should allow the design of interventions to attenuate MDs pathology without disrupting regenerative processes. In this review we will focus on the contribution of immune cells in the onset and progression of MDs, with particular emphasis on Duchenne muscular dystrophy (DMD). We will briefly summarize the current knowledge and recent advances made in our understanding of the involvement of different innate immune cells in MDs and will move on to critically evaluate the possible role of cell populations within the acquired immune response. Revisiting previous observations in the light of recent evidence will likely change our current view of the onset and progression of the disease.

Characterisation of dystrophin during development of human skeletal muscle

Development ◽  
1992 ◽  
Vol 114 (2) ◽  
pp. 395-402 ◽  
Author(s):  
A. Clerk ◽  
P.N. Strong ◽  
C.A. Sewry
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Human Skeletal Muscle ◽  
Gradual Increase ◽  
Differential Staining ◽  
Relative Molecular Mass ◽  
Adult Tissue ◽  
Dmd Gene

Dystrophin, the 427 × 10(3) Mr product of the Duchenne muscular dystrophy (DMD) gene, was studied in human foetal skeletal muscle from 9 to 26 weeks of gestation. Dystrophin could be detected from at least 9 weeks of gestation at the sarcolemmal membrane of most myotubes, though there was differential staining with antibodies raised to various regions of the protein. Dystrophin immunostaining increased and became more uniform with age and by 26 weeks of gestation there was intense sarcolemmal staining of all myotubes. On a Western blot, a doublet of smaller relative molecular mass than that seen in adult tissue was detected in all foetuses studied. There was a gradual increase in abundance of the upper band from 9 to 26 weeks, and the lower band, although present in low amounts in young foetuses, increased significantly between 20 and 26 weeks of gestation. These data indicate that there are several specific isoforms of dystrophin present in developing skeletal muscle, though the role of these is unknown.

scholarly journals Insight into the Key Points of Preeclampsia Pathophysiology: Uterine Artery Remodeling and the Role of MicroRNAs

2021 ◽  
Vol 22 (6) ◽  
pp. 3132
Author(s):  
Katarzyna Pankiewicz ◽  
Anna Fijałkowska ◽  
Tadeusz Issat ◽  
Tomasz M. Maciejewski
Keyword(s):  
Uterine Artery ◽  
Clinical Symptoms ◽  
Multiorgan Failure ◽  
Endothelial Damage ◽  
Disease Stage ◽  
Stage Model ◽  
Two Stage ◽  
Placental Perfusion ◽  
Artery Remodeling

Preeclampsia affects about 3–8% of all pregnancies. It represents a complex and multifaceted syndrome with at least several potential pathways leading to the development of disease. The main dogma in preeclampsia is the two-stage model of disease. Stage 1 (placental stage) takes place in early pregnancy and is thought to be impaired placentation due to inadequate trophoblastic invasion of the maternal spiral arteries that leads to reduced placental perfusion and release of numerous biological factors causing endothelial damage and development of acute maternal syndrome with systemic multiorgan failure (stage 2—the onset of maternal clinical symptoms, maternal stage). Recently, in the light of the vast body of evidence, two-stage model of preeclampsia has been updated with a few novel pathways leading to clinical manifestation in the second part of pregnancy. This paper reviews current state of knowledge about pathophysiology of preeclampsia and places particular focus on the recent advances in understanding of uterine artery remodeling alterations, as well as the role of microRNAs in preeclampsia.

Lokaler Journalismus und Wirtschafts-PR

2019 ◽  
Author(s):  
Felix Keldenich
Keyword(s):  
Special Functions ◽  
Personal Characteristics ◽  
Stage Model ◽  
Two Stage ◽  
Business News ◽  
Structure Dynamics ◽  
Common Practices ◽  
Individual Effects ◽  
Complex Balancing

How do local journalists perceive the PR of local businesses and how are they influenced by it? This study uses guided interviews with local German journalists to explore the role of PR in local business news. It reveals a wide variety of common practices in different editorial offices. In reference to Schimank’s approach of ‘Akteur-Struktur-Dynamiken’ (actor–structure–dynamics), influences can be assigned to what journalists want to do, what expectations they perceive and what options they have. Journalists face a complex balancing process when they deal with PR conducted in their direct neighborhood. Personal characteristics and relationships are just as important as journalistic self-conceptions and the special functions of local journalism. This book depicts the influence of PR in a two-stage model that considers both manifold factors and their individual effects.

scholarly journals DOCK3 is a dosage-sensitive regulator of skeletal muscle and Duchenne muscular dystrophy-associated pathologies

2020 ◽  
Vol 29 (17) ◽  
pp. 2855-2871
Author(s):  
Andrea L Reid ◽  
Yimin Wang ◽  
Adrienne Samani ◽  
Rylie M Hightower ◽  
Michael A Lopez ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Muscle Function ◽  
Myogenic Differentiation ◽  
Nucleotide Exchange ◽  
Dystrophic Muscle ◽  
Guanine Nucleotide Exchange ◽  
Myogenic Factors

Abstract DOCK3 is a member of the DOCK family of guanine nucleotide exchange factors that regulate cell migration, fusion and viability. Previously, we identified a dysregulated miR-486/DOCK3 signaling cascade in dystrophin-deficient muscle, which resulted in the overexpression of DOCK3; however, little is known about the role of DOCK3 in muscle. Here, we characterize the functional role of DOCK3 in normal and dystrophic skeletal muscle. Utilizing Dock3 global knockout (Dock3 KO) mice, we found that the haploinsufficiency of Dock3 in Duchenne muscular dystrophy mice improved dystrophic muscle pathologies; however, complete loss of Dock3 worsened muscle function. Adult Dock3 KO mice have impaired muscle function and Dock3 KO myoblasts are defective for myogenic differentiation. Transcriptomic analyses of Dock3 KO muscles reveal a decrease in myogenic factors and pathways involved in muscle differentiation. These studies identify DOCK3 as a novel modulator of muscle health and may yield therapeutic targets for treating dystrophic muscle symptoms.

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