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denervated muscular atrophy

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https://www.readbyqxmd.com/read/30528741/motor-unit-number-index-munix-of-hand-muscles-is-a-disease-biomarker-for-adult-spinal-muscular-atrophy
#1
René Günther, Christoph Neuwirth, Jan Christoph Koch, Paul Lingor, Nathalie Braun, Robert Untucht, Daniel Petzold, Markus Weber, Andreas Hermann
OBJECTIVE: There is still insufficient knowledge about natural history in adult spinal muscular atrophy, thus valid markers for treatment and disease monitoring are urgently needed. METHODS: We studied hand muscle innervation pattern of 38 adult genetically confirmed 5q spinal muscular atrophy (SMA) patients by the motor unit number index (MUNIX) method. Data were compared to healthy controls and amyotrophic lateral sclerosis (ALS) patients and systematically correlated to typical disease-relevant scores and other clinical as well as demographic characteristics...
November 24, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/30500392/carm1-contributes-to-skeletal-muscle-wasting-by-mediating-foxo3-activity-and-promoting-myofiber-autophagy
#2
Yuantong Liu, Jianmin Li, Yue Shang, Yan Guo, Zhenzhong Li
Coactivator-associated arginine methyltransferase 1 (CARM1) is involved in a variety of biological processes in different cell types and disease conditions, including myogenesis. However, the specific function of CARM1 in skeletal muscle wasting under pathologic conditions remains unclear. Here, we identify CARM1 as a novel participant in muscular atrophy. Increases in CARM1 protein levels correlated positively with the loss of muscle mass upon denervation in mice. Notably, the knockdown of CARM1 represses the progression of muscle wasting and the expression of the atrophy-related genes Atrogin-1 and MuRF1 in vivo and in vitro...
November 27, 2018: Experimental Cell Research
https://www.readbyqxmd.com/read/30390248/muscle-changes-during-atrophy
#3
Adrian Dumitru, Beatrice Mihaela Radu, Mihai Radu, Sanda Maria Cretoiu
Muscle atrophy typically is a direct effect of protein degradation induced by a diversity of pathophysiologic states such as disuse, immobilization, denervation, aging, sepsis, cachexia, glucocorticoid treatment, hereditary muscular disorders, cancer, diabetes and obesity, kidney and heart failure, and others. Muscle atrophy is defined by changes in the muscles, consisting in shrinkage of myofibers, changes in the types of fiber and myosin isoforms, and a net loss of cytoplasm, organelles and overall a protein loss...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/30385095/ighmbp2-mutation-associated-with-organ-specific-autonomic-dysfunction
#4
Pedro J Tomaselli, Alejandro Horga, Alexander M Rossor, Zane Jaunmuktane, Andrea Cortese, Julian C Blake, Natalia Zarate-Lopez, Henry Houlden, Mary M Reilly
Biallelic mutations in the IGHMBP2 have been associated with two distinct phenotypes: spinal muscular atrophy with respiratory distress type 1 (SMARD1) and CMT2S. We describe a patient who developed progressive muscle weakness and wasting in her upper and lower limbs from infancy. She developed respiratory involvement at age 9, eventually requiring 24-h non-invasive ventilation, and severe autonomic dysfunction restricted to the gastrointestinal tract. Neurophysiological studies at age 27 years revealed absent sensory and motor responses and severe chronic denervation changes in proximal muscles of the upper limbs...
August 29, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/30380962/sensory-neurotization-of-muscle-past-present-and-future-considerations
#5
Steven D Kozusko, Alexander J Kaminsky, Louisa C Boyd, Petros Konofaos
Research has shown that temporary innervation by a sensory neuron can provide trophic support to a denervated muscle and stave off muscular atrophy until motor neuron transfer is viable. This so called 'sensory protection' allows for improved outcomes when motor reinnervation able to occur. The theoretical benefit of sensory neurotization is hypothesized to maintain tissue architecture of the end organ due to tropic effects of stimulation. While the literature supports direct motor neurotization from 2 to 4 months post-injury, patient factors including the location of the injury and loss of nerve can preclude this therapeutic window...
October 31, 2018: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/30142977/single-muscle-fiber-proteomics-reveals-distinct-protein-changes-in-slow-and-fast-fibers-during-muscle-atrophy
#6
Franziska Lang, Solmaz Khaghani, Clara Türk, Janica Lea Wiederstein, Soraya Hölper, Tanja Piller, Leonardo Nogara, Bert Blaauw, Stefan Günther, Stefan Müller, Thomas Braun, Marcus Krüger
Skeletal muscles are composed of heterogeneous collections of fibers with different metabolic profiles. With varied neuronal innervation and fiber-type compositions, each muscle fulfils specific functions and responds differently to stimuli and perturbations. We assessed individual fibers by mass spectrometry to dissect protein changes after loss of neuronal innervation due to section of the sciatic nerve in mice. This proteomics approach enabled us to quantify ∼600 proteins per individual soleus and EDL (extensor digitorum longus) muscle fiber...
October 5, 2018: Journal of Proteome Research
https://www.readbyqxmd.com/read/30050118/denervation-activated-stat3-il-6-signalling-in-fibro-adipogenic-progenitors-promotes-myofibres-atrophy-and-fibrosis
#7
Luca Madaro, Magda Passafaro, David Sala, Usue Etxaniz, Francesca Lugarini, Daisy Proietti, Maria Vittoria Alfonsi, Chiara Nicoletti, Sole Gatto, Marco De Bardi, Ricardo Rojas-García, Lorenzo Giordani, Sara Marinelli, Vittoria Pagliarini, Claudio Sette, Alessandra Sacco, Pier Lorenzo Puri
Fibro-adipogenic progenitors (FAPs) are typically activated in response to muscle injury, and establish functional interactions with inflammatory and muscle stem cells (MuSCs) to promote muscle repair. We found that denervation causes progressive accumulation of FAPs, without concomitant infiltration of macrophages and MuSC-mediated regeneration. Denervation-activated FAPs exhibited persistent STAT3 activation and secreted elevated levels of IL-6, which promoted muscle atrophy and fibrosis. FAPs with aberrant activation of STAT3-IL-6 signalling were also found in mouse models of spinal cord injury, spinal muscular atrophy, amyotrophic lateral sclerosis (ALS) and in muscles of ALS patients...
August 2018: Nature Cell Biology
https://www.readbyqxmd.com/read/29791958/imaging-patterns-of-muscle-atrophy
#8
REVIEW
Marc-André Weber, Marcel Wolf, Mike P Wattjes
The role of muscle imaging in the diagnosis of inherited and acquired muscle diseases has gained clinical relevance. In particular, magnetic resonance imaging (MRI) is increasingly being used for diagnostic purposes, especially with its capability of whole-body musculature assessment. The assessment and quantification of muscle involvement in muscle diseases can be of diagnostic value by identifying a certain involvement pattern and thus narrowing the differential diagnosis and supporting the clinical diagnosis...
July 2018: Seminars in Musculoskeletal Radiology
https://www.readbyqxmd.com/read/29783071/mef2a-regulates-calpain-3-expression-in-l6-myoblasts
#9
Ronghua Wu, Jun Wang, Jian Yao, Zhangji Dong, Yan Liu, Mei Liu
Calpain 3 (Capn3), a skeletal muscle-specific member of the calpain family, executes some non-proteolytic functions besides its role as a Ca2+ -regulated proteolytic enzyme. Previously, we found that changes in Capn3 expression were linearly correlated with the degree of muscular atrophy following reversible sciatic nerve injury and that knockdown of Capn3 gene expression promoted myoblast differentiation. While the regulation of capn3 gene expression is interesting, transcriptional regulation of Capn3 is still unclear...
August 20, 2018: Gene
https://www.readbyqxmd.com/read/29767748/glial-activation-and-central-synapse-loss-but-not-motoneuron-degeneration-are-prevented-by-the-sigma-1-receptor-agonist-pre-084-in-the-smn2b-mouse-model-of-spinal-muscular-atrophy
#10
Clàudia Cerveró, Alba Blasco, Olga Tarabal, Anna Casanovas, Lídia Piedrafita, Xavier Navarro, Josep E Esquerda, Jordi Calderó
Spinal muscular atrophy (SMA) is characterized by the loss of α-motoneurons (MNs) with concomitant muscle denervation. MN excitability and vulnerability to disease are particularly regulated by cholinergic synaptic afferents (C-boutons), in which Sigma-1 receptor (Sig1R) is concentrated. Alterations in Sig1R have been associated with MN degeneration. Here, we investigated whether a chronic treatment with the Sig1R agonist PRE-084 was able to exert beneficial effects on SMA. We used a model of intermediate SMA, the Smn2B/- mouse, in which we performed a detailed characterization of the histopathological changes that occur throughout the disease...
July 1, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29725254/resveratrol-attenuates-denervation-induced-muscle-atrophy-due-to-the-blockade-of-atrogin-1-and-p62-accumulation
#11
Yuka Asami, Miki Aizawa, Masakazu Kinoshita, Junji Ishikawa, Kunihiro Sakuma
Decrease in activity stress induces skeletal muscle atrophy. A previous study showed that treatment with resveratrol inhibits muscular atrophy in mdx mice, a model of DMD. However, almost all studies using resveratrol supplementation have only looked at adaptive changes in the muscle weight. The present study was designed to elucidate whether the resveratrol-inducing attenuation of skeletal muscle actually reflects the adaptation of muscle fibers themselves, based on the modulation of atrogin-1- or p62-dependent signaling...
2018: International Journal of Medical Sciences
https://www.readbyqxmd.com/read/29512306/preventing-muscle-wasting-by-osteoporosis-drug-alendronate-in-vitro-and-in-myopathy-models-via-sirtuin-3-down-regulation
#12
Hsien-Chun Chiu, Chen-Yuan Chiu, Rong-Sen Yang, Ding-Cheng Chan, Shing-Hwa Liu, Chih-Kang Chiang
BACKGROUND: A global consensus on the loss of skeletal muscle mass and function in humans refers as sarcopenia and cachexia including diabetes, obesity, renal failure, and osteoporosis. Despite a current improvement of sarcopenia or cachexia with exercise training and supportive therapies, alternative and specific managements are needed to discover for whom are unable or unwilling to embark on these treatments. Alendronate is a widely used drug for osteoporosis in the elderly and postmenopausal women...
June 2018: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/29451848/mr-imaging-of-atraumatic-muscle-disorders
#13
REVIEW
Edward Smitaman, Dyan V Flores, Catalina Mejía Gómez, Mini N Pathria
Atraumatic disorders of skeletal muscles include congenital variants; inherited myopathies; acquired inflammatory, infectious, or ischemic disorders; neoplastic diseases; and conditions leading to muscle atrophy. These have overlapping appearances at magnetic resonance (MR) imaging and are challenging for the radiologist to differentiate. The authors organize muscle disorders into four MR imaging patterns: (a) abnormal anatomy with normal signal intensity, (b) edema/inflammation, (c) mass, and (d) atrophy, highlighting each of their key clinical and imaging findings...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29440993/increasing-agrin-function-antagonizes-muscle-atrophy-and-motor-impairment-in-spinal-muscular-atrophy
#14
Marina Boido, Elena De Amicis, Valeria Valsecchi, Marco Trevisan, Ugo Ala, Markus A Ruegg, Stefan Hettwer, Alessandro Vercelli
Spinal muscular atrophy (SMA) is a pediatric genetic disease, characterized by motor neuron (MN) death, leading to progressive muscle weakness, respiratory failure, and, in the most severe cases, to death. Abnormalities at the neuromuscular junction (NMJ) have been reported in SMA, including neurofilament (NF) accumulation at presynaptic terminals, immature and smaller than normal endplates, reduced transmitter release, and, finally, muscle denervation. Here we have studied the role of agrin in SMAΔ7 mice, the experimental model of SMAII...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29302496/selective-pectoralis-major-muscle-denervation-in-breast-reconstruction-a-technical-modification-for-more-effective-and-cosmetic-results
#15
Marco Bernini, Donato Casella, Carlo Mariotti
The use of pectoralis major muscle (PMM) in breast reconstruction has been a mainstay for decades. In recent years, although, a novel approach, the so-called subcutaneous or pre-pectoral breast reconstruction, has been introduced advocating the advantages of sparing the pectoralis muscle. Such advantages include more natural implant ptosis and appearance, significant reduction of capsular contracture and also avoidance of implant animation and animation deformities. These are all drawbacks that the use of muscles in implant coverage inevitably involves overtime...
December 2017: Gland Surgery
https://www.readbyqxmd.com/read/29272405/selective-vulnerability-in-neuronal-populations-in-nmd-smard1-mice
#16
Eric Villalón, Monir Shababi, Rachel Kline, Zachary C Lorson, Kyra M Florea, Christian L Lorson
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is an autosomal recessive motor neuron disease causing distal limb muscle atrophy that progresses proximally and is accompanied by diaphragmatic paralysis. Neuromuscular junction (NMJ) alterations have been reported in muscles of SMARD1 model mice, known as nmd mice, with varying degrees of severity, suggesting that different muscles are specifically and selectively resistant or susceptible to denervation. To evaluate the extent of NMJ pathology in a broad range of muscles, a panel of axial and appendicular muscles were isolated and immunostained from nmd mice...
February 15, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/28931824/boosted-regeneration-and-reduced-denervated-muscle-atrophy-by-neuroheal-in-a-pre-clinical-model-of-lumbar-root-avulsion-with-delayed-reimplantation
#17
David Romeo-Guitart, Joaquim Forés, Xavier Navarro, Caty Casas
The "gold standard" treatment of patients with spinal root injuries consists of delayed surgical reconnection of nerves. The sooner, the better, but problems such as injury-induced motor neuronal death and muscle atrophy due to long-term denervation mean that normal movement is not restored. Herein we describe a preclinical model of root avulsion with delayed reimplantation of lumbar roots that was used to establish a new adjuvant pharmacological treatment. Chronic treatment (up to 6 months) with NeuroHeal, a new combination drug therapy identified using a systems biology approach, exerted long-lasting neuroprotection, reduced gliosis and matrix proteoglycan content, accelerated nerve regeneration by activating the AKT pathway, promoted the formation of functional neuromuscular junctions, and reduced denervation-induced muscular atrophy...
September 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28647557/kcc3-loss-of-function-contributes-to-andermann-syndrome-by-inducing-activity-dependent-neuromuscular-junction-defects
#18
Melissa Bowerman, Céline Salsac, Véronique Bernard, Claire Soulard, Annie Dionne, Emmanuelle Coque, Salim Benlefki, Pascale Hince, Patrick A Dion, Gillian Butler-Browne, William Camu, Jean-Pierre Bouchard, Eric Delpire, Guy A Rouleau, Cédric Raoul, Frédérique Scamps
Loss-of-function mutations in the potassium-chloride cotransporter KCC3 lead to Andermann syndrome, a severe sensorimotor neuropathy characterized by areflexia, amyotrophy and locomotor abnormalities. The molecular events responsible for axonal loss remain poorly understood. Here, we establish that global or neuron-specific KCC3 loss-of-function in mice leads to early neuromuscular junction (NMJ) abnormalities and muscular atrophy that are consistent with the pre-synaptic neurotransmission defects observed in patients...
October 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28556540/sustained-nf%C3%AE%C2%BAb-inhibition-improves-insulin-sensitivity-but-is-detrimental-to-muscle-health
#19
Ning Zhang, Joseph M Valentine, You Zhou, Mengyao E Li, Yiqiang Zhang, Arunabh Bhattacharya, Michael E Walsh, Katherine E Fischer, Steven N Austad, Pawel Osmulski, Maria Gaczynska, Steven E Shoelson, Holly Van Remmen, Hung I Chen, Yidong Chen, Hanyu Liang, Nicolas Musi
Older adults universally suffer from sarcopenia and approximately 60-70% are diabetic or prediabetic. Nonetheless, the mechanisms underlying these aging-related metabolic disorders are unknown. NFκB has been implicated in the pathogenesis of several aging-related pathologies including sarcopenia and type 2 diabetes and has been proposed as a target against them. NFκB also is thought to mediate muscle wasting seen with disuse, denervation, and some systemic diseases (e.g., cancer, sepsis). We tested the hypothesis that lifelong inhibition of the classical NFκB pathway would protect against aging-related sarcopenia and insulin resistance...
August 2017: Aging Cell
https://www.readbyqxmd.com/read/28520806/als-skeletal-muscle-shows-enhanced-tgf-%C3%AE-signaling-fibrosis-and-induction-of-fibro-adipogenic-progenitor-markers
#20
David Gonzalez, Osvaldo Contreras, Daniela L Rebolledo, Juan Pablo Espinoza, Brigitte van Zundert, Enrique Brandan
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease in which upper and lower motoneurons degenerate leading to muscle wasting, paralysis and eventually death from respiratory failure. Several studies indicate that skeletal muscle contributes to disease progression; however the molecular mechanisms remain elusive. Fibrosis is a common feature in skeletal muscle under chronic damage conditions such as those caused by muscular dystrophies or denervation. However, the exact mechanisms of fibrosis induction and the cellular bases of this pathological response are unknown...
2017: PloS One
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