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Muscle atrophy

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https://www.readbyqxmd.com/read/29783071/mef2a-regulates-calpain-3-expression-in-l6-myoblasts
#1
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...
May 18, 2018: Gene
https://www.readbyqxmd.com/read/29782610/castration-induces-satellite-cell-activation-that-contributes-to-skeletal-muscle-maintenance
#2
Alanna Klose, Wenxuan Liu, Nicole D Paris, Sophie Forman, John J Krolewski, Kent L Nastiuk, Joe V Chakkalakal
Background: Sarcopenia, the age-related loss of skeletal muscle, is a side effect of androgen deprivation therapy (ADT) for prostate cancer patients. Resident stem cells of skeletal muscle, satellite cells (SCs), are an essential source of progenitors for the growth and regeneration of skeletal muscle. Decreased androgen signaling and deficits in the number and function of SCs are features of aging. Although androgen signaling is known to regulate skeletal muscle, the cellular basis for ADT-induced exacerbation of sarcopenia is unknown...
2018: JCSM Rapid Commun
https://www.readbyqxmd.com/read/29781585/group-i-paks-support-muscle-regeneration-and-counteract-cancer-associated-muscle-atrophy
#3
Andrea Cerquone Perpetuini, Andrea David Re Cecconi, Michela Chiappa, Giulia Benedetta Martinelli, Claudia Fuoco, Giovanni Desiderio, Luisa Castagnoli, Cesare Gargioli, Rosanna Piccirillo, Gianni Cesareni
BACKGROUND: Skeletal muscle is characterized by an efficient regeneration potential that is often impaired during myopathies. Understanding the molecular players involved in muscle homeostasis and regeneration could help to find new therapies against muscle degenerative disorders. Previous studies revealed that the Ser/Thr kinase p21 protein-activated kinase 1 (Pak1) was specifically down-regulated in the atrophying gastrocnemius of Yoshida hepatoma-bearing rats. In this study, we evaluated the role of group I Paks during cancer-related atrophy and muscle regeneration...
May 21, 2018: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/29781327/the-progress-of-aav-mediated-gene-therapy-in-neuromuscular-disorders
#4
Sara Aguti, Alberto Malerba, Haiyan Zhou
The well-defined genetic causes and monogenetic nature of many neuromuscular disorders, including Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA), present gene therapy as a prominent therapeutic approach. The novel variants of adeno-associated virus (AAV) can achieve satisfactory transduction efficiency of exogenous genes through the central nervous system and body-wide in skeletal muscle. Areas covered: In this review, we summarize the strategies of AAV gene therapy that are currently under preclinical and clinical evaluation for the treatment of degenerative neuromuscular disorders, with a focus on diseases such as DMD and SMA...
May 20, 2018: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/29780939/sixth-cranial-nerve-palsy-and-ipsilateral-trigeminal-neuralgia-caused-by-vertebrobasilar-dolichoectasia
#5
Thong Pham, Jeffrey Wesolowski, Jonathan D Trobe
Purpose: To report an unusual case of vertebrobasilar dolichoectasia causing both trigeminal neuralgia and ipsilateral sixth cranial nerve palsy. Observations: A patient had undergone surgical decompression of trigeminal neuralgia caused by dolichoectatic vertebral and basilar arteries years before presenting with ipsilateral sixth nerve palsy. Brain MRI showed deviant vertebrobasilar arteries that presumably now compressed the sixth cranial nerve. The unaffected left sixth cranial nerve was visible on MRI, but the affected right sixth cranial nerve was not...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29780606/trismus-masticatory-myositis-and-antibodies-against-type-2m-fibers-in-a-mixed-breed-cat
#6
Stanley W Blazejewski, G Diane Shelton
Case summary: A 1-year-old male neutered mixed breed cat presented with a 2 month history of inability to fully open the mouth when yawning and decreased ability to prehend food. Physical examination revealed severe bilaterally symmetrical masticatory muscle atrophy, a restricted vertical mandibular range of motion of 11-12 mm, and a normal body condition score. Skull radiography was normal. A canine ELISA system against unique masticatory muscle fibers (2M antibody titer), was positive at 1:1000 (reference interval <1:100 in dogs, and was <1:100 using serum from five archived normal cats), indicating the presence of cross-reacting antibodies...
January 2018: JFMS Open Reports
https://www.readbyqxmd.com/read/29779597/amyotrophic-lateral-sclerosis-and-the-respiratory-system
#7
REVIEW
Andrew T Braun, Candelaria Caballero-Eraso, Noah Lechtzin
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that always affects the respiratory muscles. It is characterized by degeneration of motor neurons in the brain and spinal cord. Respiratory complications are the most common causes of death in ALS and typically occur within 3 to 5 years of diagnosis. Because ALS affects both upper and lower motor neurons, it causes hyperreflexia, spasticity, muscle fasciculations, muscle atrophy, and weakness. It ultimately progresses to functional quadriplegia...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29777277/impact-of-muscle-atrophy-on-bone-metabolism-and-bone-strength-implications-for-muscle-bone-crosstalk-with-aging-and-disuse
#8
REVIEW
T Bettis, B-J Kim, M W Hamrick
Bone fractures in older adults are often preceded by a loss of muscle mass and strength. Likewise, bone loss with prolonged bed rest, spinal cord injury, or with exposure to microgravity is also preceded by a rapid loss of muscle mass. Recent studies using animal models in the setting of hindlimb unloading or botulinum toxin (Botox) injection also reveal that muscle loss can induce bone loss. Moreover, muscle-derived factors such as irisin and leptin can inhibit bone loss with unloading, and knockout of catabolic factors in muscle such as the ubiquitin ligase Murf1 or the myokine myostatin can reduce osteoclastogenesis...
May 18, 2018: Osteoporosis International
https://www.readbyqxmd.com/read/29774167/complete-hypokalemic-quadriparesis-as-a-first-presentation-of-sj%C3%A3-gren-syndrome
#9
Jason An, Branko Braam
Rationale: We hope to increase awareness that hypokalemic paralysis may be the first presentation of Sjögren syndrome, for which potassium-sparing diuretics can be an effective adjunct to potassium replenishment. Presenting concerns: A 73-year-old female presented to a peripheral hospital with quadriparesis and a critically low serum potassium of 1.6 mmol/L with U waves on the electrocardiogram (ECG). The initial arterial blood gas showed a pH of 7.19, bicarbonate of 13 mEq/L, and a CO2 of 35 mm Hg...
2018: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/29772368/volumetric-muscle-measurements-indicate-significant-muscle-degeneration-in-single-level-disc-herniation-patients
#10
Kaan Yaltırık, Burhan Oral Güdü, Yüksel Işık, Çiğdem Altunok, Ufuk Tipi, Başar Atalay
INTRODUCTION: There is a strong relationship between lower back pain and paraspinal muscle atrophy. In this study, we aimed to investigate the prevalence of lumbar paravertebral muscle atrophy in patients with and without single level disc herniation. MATERIALS AND METHODS: The 110 retrospectively analyzed patients in this study were divided into two groups. Group A included 55 patients with radiologically confirmed single level disc herniation with back pain and radiculopathy...
May 14, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29772095/effect-of-specific-exercise-strategy-on-strength-and-proprioception-in-volleyball-players-with-infraspinatus-muscle-atrophy
#11
José Inácio Salles, Victor Rodrigues Amaral Cossich, João Matheus Guimarães, Geraldo Motta Filho, Dylan Morrissey
Infraspinatus muscle atrophy is common in professional volleyball players, but it is unclear whether commonly observed strength and proprioception deficits can be reversed with training. Fifty-four participants were recruited into an infraspinatus atrophy group (IAG, n = 18) and a non-atrophy group (NAG, n=18) of elite volleyballers plus a healthy non-athletic control group (CG, n = 18). IAG were trained with a progressive, specific shoulder external rotator strengthening routine for 32 sessions over 8 weeks...
May 17, 2018: Scandinavian Journal of Medicine & Science in Sports
https://www.readbyqxmd.com/read/29770363/the-multifaceted-clinical-presentation-of-vcp-proteinopathy-in-a-greek-family
#12
George K Papadimas, George P Paraskevas, Thomas Zambelis, Chrisostomos Karagiaouris, Mara Bourbouli, Anastasia Bougea, Maggie C Walter, Nicolas U Schumacher, Sabine Krause, Elisabeth Kapaki
VCP-proteinopathy is a multisystem neurodegenerative disorder caused by mutations in valosin containing protein. Here, we report the first Greek case of VCP-proteinopathy in a 62 year old patient with a slowly progressing muscular weakness since his mid-40s and a severe deterioration during the last year. He also manifested dementia with prominent neuropsychiatric symptoms, including aggression, apathy, palilalia and obsessions. Brain MRI revealed frontal atrophy, while muscle MRI showed diffuse muscle atrophy...
December 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29770362/study-of-anti-m%C3%A3-llerian-hormone-levels-in-patients-with-myotonic-dystrophy-type-1-preliminary-results
#13
Manuela Ergoli, Massimo Venditti, Raffaele Dotolo, Esther Picillo, Sergio Minucci, Luisa Politano
Myotonic dystrophy type 1 is a multisystemic disorder characterized by myotonia, muscle weakness and involvement of several organs and apparatus such as heart, lungs, eye, brain and endocrine system. Hypogonadism and reproductive abnormalities are frequently reported. A progressive testicular atrophy occurs in about 80% in the affected males leading to Leydig cell hyperproliferation and elevated basal follicle stimulating hormone (FSH) levels. Anti-Müllerian hormone (AMH) - a dimeric glycoprotein belonging to the super-family of transforming grow factor beta (TGF-beta) - is the earliest Sertoli cell hormone secreted in males and, together with inhibin B and FSH, is an important indicator of Sertoli cell function...
December 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29768017/the-signaling-network-resulting-in-ventilator-induced-diaphragm-dysfunction
#14
Huibin Tang, Joseph B Shrager
Mechanical ventilation (MV) is a life-saving measure for those incapable of adequately ventilating or oxygenating without assistance. Unfortunately, even brief periods of MV result in diaphragm weakness (i.e., "ventilator-induced diaphragm dysfunction" - VIDD) that may render it difficult to wean the ventilator. Prolonged MV is associated with cascading complications and is a strong risk factor for death. Thus, prevention of VIDD may have a dramatic impact on mortality rates. Here, we summarized the current understanding of the pathogenic events underlying VIDD...
May 16, 2018: American Journal of Respiratory Cell and Molecular Biology
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
#15
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...
May 14, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29766941/idiopathic-inflammatory-myopathies-in-adults-a-comparative-study-of-bohan-and-peter-and-european-neuromuscular-center-2004-criteria
#16
Sundaram Challa, Saumya Jakati, Megha S Uppin, Meena A Kannan, Rajasekhar Liza, M K Murthy Jagarlapudi
Background: Bohan and Peter criteria are widely used for the diagnosis of idiopathic inflammatory myopathies (IIMs). Recently, European Neuromuscular Center (ENMC) formulated criteria to identify subgroups of IIMs. Aim: To compare the two diagnostic criteria in adult IIMs. Materials and Methods: This was a retrospective review of case records of histologically confirmed IIMs in adults between January 2014 and May 2015. Both the Bohan and Peter, and ENMC 2004 criteria were applied in the same group of patients to subgroup the IIMs...
May 2018: Neurology India
https://www.readbyqxmd.com/read/29765879/quantitative-measurement-of-muscle-atrophy-and-fat-infiltration-of-the-supraspinatus-muscle-using-ultrasonography-after-arthroscopic-rotator-cuff-repair
#17
Yong Ki Kim, Eun Seok Choi, Keon Tae Kim, Jung Ro Yoon, Sang Han Chae
Objective: To investigate the utility of ultrasonography to objectively examine morphological changes (i.e., muscle atrophy and fatty infiltration) of the supraspinatus muscle. Methods: Thirty-four patients were prospectively enrolled in this study. The degrees of muscle atrophy and fat infiltration were measured using ultrasonography 3-4 months after arthroscopic supraspinatus tendon repair. Shoulder function (i.e., shoulder active range of motion, visual analogue scale, and constant score) was examined...
April 2018: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/29764991/a-dgk%C3%AE-foxo-ubiquitin-proteolytic-axis-controls-fiber-size-during-skeletal-muscle-remodeling
#18
Jae-Sung You, Matthew S Dooley, Chan-Ran Kim, Eui-Jun Kim, Wei Xu, Craig A Goodman, Troy A Hornberger
Skeletal muscle rapidly remodels in response to various stresses, and the resulting changes in muscle mass profoundly influence our health and quality of life. We identified a diacylglycerol kinase ζ (DGKζ)-mediated pathway that regulated muscle mass during remodeling. During mechanical overload, DGKζ abundance was increased and required for effective hypertrophy. DGKζ not only augmented anabolic responses but also suppressed ubiquitin-proteasome system (UPS)-dependent proteolysis. We found that DGKζ inhibited the transcription factor FoxO that promotes the induction of the UPS...
May 15, 2018: Science Signaling
https://www.readbyqxmd.com/read/29763467/natural-disease-history-of-the-dy2j-mouse-model-of-laminin-%C3%AE-2-merosin-deficient-congenital-muscular-dystrophy
#19
S Pasteuning-Vuhman, K Putker, C L Tanganyika-de Winter, J W Boertje-van der Meulen, L van Vliet, M Overzier, J J Plomp, A Aartsma-Rus, M van Putten
Merosin deficient congenital muscular dystrophy 1A (MDC1A) is a very rare autosomal recessive disorder caused by mutations in the LAMA2 gene leading to severe and progressive muscle weakness and atrophy. Although over 350 causative mutations have been identified for MDC1A, no treatment is yet available. There are many therapeutic approaches in development, but the lack of natural history data of the mouse model and standardized outcome measures makes it difficult to transit these pre-clinical findings to clinical trials...
2018: PloS One
https://www.readbyqxmd.com/read/29761850/unexpected-regulation-pattern-of-the-ikk%C3%AE-nf-%C3%AE%C2%BAb-murf1-pathway-with-remarkable-muscle-plasticity-in-the-daurian-ground-squirrel-spermophilus-dauricus
#20
Yanhong Wei, Lingchen Gong, Weiwei Fu, Shenhui Xu, Zhe Wang, Jie Zhang, Er Ning, Hui Chang, Huiping Wang, Yunfang Gao
As a typical hibernator, the Daurian ground squirrel (Spermophilus dauricus) spends considerable time in a state of reduced activity with prolonged fasting. Despite this, they experience little muscle atrophy and have thus become an interesting anti-disuse muscle atrophy model. The IKKβ/NF-κB signaling pathway is significant to muscle atrophy due to the protein degradation resulting from the upregulation of the E3 ubiquitin ligase MuRF1. The current study showed that the IKKβ/NF-κB signaling pathway and MuRF1 maintained relatively steady mRNA and protein expression levels, with little muscle atrophy observed in the soleus (slow-twitch, SOL) or extensor digitorum longus (fast-twitch, EDL) during hibernation (HIB); however, mRNA expression significantly increased in the SOL and EDL muscle during interbout arousal (IBA), as did the MuRF1 mRNA level in the SOL and MuRF1 protein level in the EDL...
May 15, 2018: Journal of Cellular Physiology
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