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(Muscle) AND (Atrophy or wasting or degradation or breakdown or disuse or immobilization or loss)

Samantha LoRusso, Benjamin Weiner, W David Arnold
Myotonic dystrophy is an autosomal dominant muscular dystrophy not only associated with muscle weakness, atrophy, and myotonia but also prominent multisystem involvement. There are 2 similar, but distinct, forms of myotonic dystrophy; type 1 is caused by a CTG repeat expansion in the DMPK gene, and type 2 is caused by a CCTG repeat expansion in the CNBP gene. Type 1 is associated with distal limb, neck flexor, and bulbar weakness and results in different phenotypic subtypes with variable onset from congenital to very late-onset as well as variable signs and symptoms...
October 18, 2018: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Nobuhiko Nakamura, Soranobu Ninomiya, Takuro Matsumoto, Hiroshi Nakamura, Junichi Kitagawa, Makoto Shiraki, Takeshi Hara, Masahito Shimizu, Hisashi Tsurumi
Skeletal muscle atrophy and loss of adipose tissue, referred to as sarcopenia and adipopenia, respectively, are often observed in cancer patients. We investigated the impact of sarcopenia and adipopenia on clinical outcomes in 90 adult patients with newly diagnosed acute myeloid leukemia (AML) who received induction chemotherapy. Computed tomography (CT) before treatment revealed sarcopenia in 39 patients (43%) and adipopenia in 35 patients (39%). We analyzed the treatment efficacy of induction chemotherapy and survival outcomes...
October 19, 2018: Annals of Hematology
Osamah Alwalid, Joyman Makamure, Qi-Guang Cheng, Wen-Jun Wu, Chao Yang, Elham Samran, Ping Han, Hui-Min Liang
Klippel-Trénaunay syndrome (KTS) is a rare angio-osteo-hypertrophic syndrome characterized by vascular malformations, soft tissue and/or bone hypertrophy, and varicose veins. For the purpose of describing the imaging findings and elucidating the role of medical imaging in the diagnosis and assessment of patient with KTS, we have reviewed the imaging data of 14 KTS patients. The imaging features on different imaging modalities were analyzed. Unilateral lower limb involvement was evident in 71% of cases (n=10) and bilateral but asymmetric lower limb involvement in the remaining 29% of cases (n=4)...
October 2018: Current medical science
Akio Sakamoto, Ryuzo Arai, Takeshi Okamoto, Yosuke Yamada, Hodaka Yamakado, Shuichi Matsuda
BACKGROUND Ischemic fasciitis is a rare condition that occurs in debilitated and immobilized individuals, usually overlying bony protuberances. Because the histology shows a pseudosarcomatous proliferation of atypical fibroblasts, and because the lesion can increase in size, ischemic fasciitis can mimic sarcoma. Beta-propeller protein-associated neurodegeneration (BPAN) arises in infancy and is due to mutations in the WDR45 gene on the X chromosome. BPAN results in progressive symptoms of dystonia, Parkinsonism, and dementia once the individual reaches adolescence or early adulthood, and is usually fatal before old age...
October 20, 2018: American Journal of Case Reports
Le Xu, Venkat R Chirasani, Jordan S Carter, Daniel A Pasek, Nikolay V Dokholyan, Naohiro Yamaguchi, Gerhard Meissner
Cryo-electron micrograph studies recently have identified a Ca2+-binding site in the 2,200-kDa ryanodine receptor ion channel (RyR1) in skeletal muscle.[MS2]  To clarify the role of this site in regulating RyR1 activity, here we applied mutational, electrophysiological, and computational methods. Three amino acid residues that interact directly with Ca2+ were replaced, and these RyR1 variants were expressed in HEK293 cells. Single-site RyR1-E3893Q, E3893V, E3967Q, E3967V, and T5001A variants and double-site RyR1-E3893Q/E3967Q and E3893V/E3967V variants displayed cellular Ca2+ release in response to caffeine, which indicated that they retained functionality as caffeine-sensitive, Ca2+-conducting channels in the HEK293 cell system...
October 19, 2018: Journal of Biological Chemistry
Wenjun Hu, Zeyuan Ru, Wen Xiao, Zhiyong Xiong, Cheng Wang, Changfei Yuan, Xiaoping Zhang, Hongmei Yang
Cancer-associated cachexia (CAC) is a disorder characterized by unintended weight loss due to skeletal muscle wasting and adipose tissue loss. Although muscle atrophy in this condition has been well studied, the mechanisms underlying adipose tissue loss, which include browning, have not been investigated in detail. In this respect, though recent studies have shown that exosomes from cancer cells can promote lipolysis, the link between exosomes from cancer cells and CAC has not been clearly established. In this study, we investigate if exosomes from Lewis lung carcinoma (LLC) cells can induce lipolysis in vitro (in 3T3-L1 adipocytes) and in vivo (in LLC tumor-bearing mice)...
October 16, 2018: Biochemical and Biophysical Research Communications
Yen-Jung Chou, Jiunn-Jye Chuu, Yi-Jen Peng, Yu-Hsuan Cheng, Chin-Hsien Chang, Chieh-Min Chang, Hsia-Wei Liu
BACKGROUND: Osteoarthritis (OA) is a slow progressing, degenerative disorder of the synovial joints. Guilu Erxian Glue (GEG) is a multi-component Chinese herbal remedy with long-lasting favorable effects on several conditions, including articular pain and muscle strength in elderly men with knee osteoarthritis. The present study aimed to identify the effects of Guilu Erxian Paste (GE-P) and Liquid (GE-L) extracted from Guilu Erxian Glue in anterior cruciate ligament transection (ACLT)-induced osteoarthritis mice, and to compare the effectiveness of different preparations on knee cartilage degeneration during the progression of osteoarthritis...
October 19, 2018: Journal of Orthopaedic Surgery and Research
Rana Yadak, Max V Boot, Niek P van Til, Dominique Cazals-Hatem, Armin Finkenstedt, Elly Bogaerts, Irenaeus F de Coo, Marianna Bugiani
BACKGROUND: Gastrointestinal complications are the main cause of death in patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). Available treatments often restore biochemical homeostasis, but fail to cure gastrointestinal symptoms. METHODS: We evaluated the small intestine neuromuscular pathology of an untreated MNGIE patient and two recipients of hematopoietic stem cells, focusing on enteric neurons and glia. Additionally, we evaluated the intestinal neuromuscular pathology in a mouse model of MNGIE treated with hematopoietic stem cell gene therapy...
October 19, 2018: BMC Gastroenterology
Marcela M Blascke de Mello, Juliana M Parente, Richard Schulz, Michele M Castro
Hypertension is characterized by maladaptive vascular remodeling and enhanced oxidative stress in the vascular wall. Peroxynitrite may directly activate latent matrix metalloproteinase (MMP)-2 in vascular smooth muscle cells (VSMC) by its S-glutathiolation. MMP-2 may then proteolyze calponin-1 in aortas from hypertensive animals, which stimulates VSMC proliferation and medial hypertrophy. Calponin-1 is an intracellular protein which helps to maintain VSMC in their differentiated (contractile) phenotype. The present study therefore investigated whether aortic MMP-2 activity is increased by oxidative stress in early hypertension and then contributes to hypertrophic arterial remodeling by reducing the levels of calponin-1...
October 16, 2018: Vascular Pharmacology
Matthew F Jacques, Gladys L Onambele-Pearson, Neil D Reeves, Georgina K Stebbings, Jonathon Smith, Christopher I Morse
BACKGROUND: Muscular dystrophy (MD) is characterized by progressive muscle wasting and weakness, yet few comparisons to non-MD controls (CTRL) of muscle strength and size in this adult population exist. Physical activity (PA) is promoted to maintain health and muscle strength within MD; however, PA reporting in adults with MD is limited to recall data, and its impact on muscle strength is seldom explored. METHODS: This study included 76 participants: 16 non-MD (CTRL, mean age 35...
October 19, 2018: Journal of Cachexia, Sarcopenia and Muscle
Hye Rim Suh, Eui Ho Park, Sun Wook Moon, Ji Won Kim, Hwi Young Cho, Hee Chul Han
INTRODUCTION: Lengthened immobilization may prevent muscle shortening, and help maintain normal muscle length. However, its apoptotic effects remain unclear. We evaluated the effects of long-term immobilization on apoptotic proteins. METHODS: Rat soleus muscles were immobilized by casting in a neutral (NEUT) or lengthened (LENG) position for 21 days. We evaluated dynamic weight load and muscle atrophy following the 21-day period using hematoxylin and eosin staining...
October 19, 2018: Muscle & Nerve
Kevin L Shimkus, Leonard S Jefferson, Bradley S Gordon, Scot R Kimball
We recently reported results showing that cast immobilization of a rat hindlimb rapidly leads to development of anabolic resistance as demonstrated by failure of oral leucine administration to activate the mechanistic target of rapamycin complex 1 (mTORC1) and stimulate protein synthesis in the soleus muscle. The goal of this study was to assess the possible contribution of several mTORC1 regulatory proteins to the development of anabolic resistance. To accomplish this, 14-week-old male C57BL/6 mice (n = 21) were subjected to unilateral cast immobilization of the hindlimb for either 1 or 3 days, and the immobilized limb was compared to its contralateral control...
October 2018: Physiological Reports
Nuria Carrillo, May C Malicdan, Marjan Huizing
GNE myopathy, previously known as hereditary inclusion body myopathy (HIBM), or Nonaka myopathy, is a rare autosomal recessive muscle disease characterized by progressive skeletal muscle atrophy. It has an estimated prevalence of 1 to 9:1,000,000. GNE myopathy is caused by mutations in the GNE gene which encodes the rate-limiting enzyme of sialic acid biosynthesis. The pathophysiology of the disease is not entirely understood, but hyposialylation of muscle glycans is thought to play an essential role. The typical presentation is bilateral foot drop caused by weakness of the anterior tibialis muscles with onset in early adulthood...
October 18, 2018: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Martin Flück, Claudio Viecelli, Andreas M Bapst, Stephanie Kasper, Paola Valdivieso, Martino V Franchi, Severin Ruoss, Jean-Marc Lüthi, Martin Bühler, Helgard Claassen, Hans Hoppeler, Christian Gerber
We investigated molecular and cellular parameters which set metabolic and mechanical functioning of knee extensor muscles in the operated and contralateral control leg of 9 patients with a chronically insufficient anterior cruciate ligament (ACL; 26.6 ± 8.3 years, 8 males, 1 female) after open reconstructive surgery (week 0), after ambulant physiotherapy under cast immobilization (week 9), succeeding rehabilitation training (up to week 26), and subsequent voluntary physical activity (week 260). Clinical indices of knee function in the operated leg were improved at 52 weeks and remained at a comparable level at week 260...
2018: Frontiers in Physiology
Mary F O'Leary, Graham R Wallace, Edward T Davis, David P Murphy, Thomas Nicholson, Andrew J Bennett, Kostas Tsintzas, Simon W Jones
Adiposity and adipokines are implicated in the loss of skeletal muscle mass with age and in several chronic disease states. The aim of this study was to determine the effects of human obese and lean subcutaneous adipose tissue secretome on myogenesis and metabolism in skeletal muscle cells derived from both young (18-30 yr) and elderly (>65 yr) individuals. Obese subcutaneous adipose tissue secretome impaired the myogenesis of old myoblasts but not young myoblasts. Resistin was prolifically secreted by obese subcutaneous adipose tissue and impaired myotube thickness and nuclear fusion by activation of the classical NFκB pathway...
October 18, 2018: Scientific Reports
Natzi Sakalihasan, Jean-Baptiste Michel, Athanasios Katsargyris, Helena Kuivaniemi, Jean-Olivier Defraigne, Alain Nchimi, Janet T Powell, Koichi Yoshimura, Rebecka Hultgren
An abdominal aortic aneurysm (AAA) is a localized dilatation of the infrarenal aorta. AAA is a multifactorial disease, and genetic and environmental factors play a part; smoking, male sex and a positive family history are the most important risk factors, and AAA is most common in men >65 years of age. AAA results from changes in the aortic wall structure, including thinning of the media and adventitia due to the loss of vascular smooth muscle cells and degradation of the extracellular matrix. If the mechanical stress of the blood pressure acting on the wall exceeds the wall strength, the AAA ruptures, causing life-threatening intra-abdominal haemorrhage - the mortality for patients with ruptured AAA is 65-85%...
October 18, 2018: Nature Reviews. Disease Primers
Dhanushka N P Munkanatta Godage, Garrett C VanHecke, Kusal T G Samarasinghe, Han-Zhong Feng, Mark Hiske, Joshua Holcomb, Zhe Yang, Jian-Ping Jin, Charles S Chung, Young-Hoon Ahn
Reactive oxygen species (ROS) contribute to the etiology of multiple muscle-related diseases. There is emerging evidence that cellular stress can lead to destabilization of sarcomeres, the contractile unit of muscle. However, it is incompletely understood how cellular stress induces structural destabilization of sarcomeres. Here we report that glutathionylation of SMYD2 contributes to a loss of myofibril integrity and degradation of sarcomeric proteins mediated by MMP-2 and calpain 1. We used a clickable glutathione approach in a cardiomyocyte cell line and found selective glutathionylation of SMYD2 at Cys13...
October 18, 2018: Nature Communications
Christopher Grunseich, Ram Miller, Therese Swan, David J Glass, Mohamed El Mouelhi, Mara Fornaro, Olivier Petricoul, Igor Vostiar, Ronenn Roubenoff, Matthew N Meriggioli, Angela Kokkinis, Robert D Guber, Maher S Budron, John Vissing, Gianni Soraru, Tahseen Mozaffar, Albert Ludolph, John T Kissel, Kenneth H Fischbeck
BACKGROUND: Spinal and bulbar muscular atrophy is an X-linked neuromuscular disease caused by CAG repeat expansion in the androgen receptor gene. Patients with this disease have low concentrations of insulin-like growth factor-1 (IGF-1), and studies of overexpression and administration of IGF-1 showed benefit in a transgenic model; thus the IGF-1 pathway presents as a potential treatment target. We assessed safety, tolerability, and preliminary efficacy of BVS857, an IGF-1 mimetic, in patients with spinal and bulbar muscular atrophy...
October 15, 2018: Lancet Neurology
Jacob M Babu, Saisanjana Kalagara, Wesley Durand, Valentin Antoci, Matthew E Deren, Eric Cohen
BACKGROUND: Sarcopenia, an age-related loss of muscle mass and function, has been previously linked to an increased risk of morbidity, mortality, and infection after a variety of surgical procedures. This study is the first to evaluate the impact of the psoas-lumbar vertebral index (PLVI), a validated marker for central sarcopenia, on determining post-arthroplasty infection status. METHODS: This is a case-control, retrospective review of 30 patients with prosthetic joint infection (PJI) diagnosed by the Musculoskeletal Infection Society criteria compared to 69 control patients who underwent a total hip or knee arthroplasty...
September 20, 2018: Journal of Arthroplasty
Qinxiu Sun, Fangda Sun, Xiufang Xia, Honghua Xu, Baohua Kong
This study investigated the impact of ultrasound-assisted immersion freezing (UIF), air freezing (AF), and immersion freezing (IF) on the ice crystal size, protein thermal stability, and physicochemical properties of common carp (Cyprinus carpio) muscle during frozen storage. UIF samples had smaller ice crystals throughout the storage period than AF and IF samples did, which led to less damage to the muscle tissue. Low-field nuclear magnetic resonance analysis revealed that UIF reduced the mobility and loss of immobilized and free water...
October 6, 2018: Ultrasonics Sonochemistry
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