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Muscle atrophy

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https://www.readbyqxmd.com/read/28815909/proximal-neuropathy-and-associated-skeletal-muscle-changes-resembling-denervation-atrophy-in-hind-limbs-of-chronic-hypoglycaemic-rats
#1
Vivi F H Jensen, Anne-Marie Molck, Henrik Soeborg, Jette Nowak, Melissa Chapman, Jens Lykkesfeldt, Ingrid B Bogh
Peripheral neuropathy is one of the most common complications of diabetic hyperglycaemia. Insulin-induced hypoglycaemia (IIH) might potentially exacerbate or contribute to neuropathy as hypoglycaemia also causes peripheral neuropathy. In rats, IIH induces neuropathy associated with skeletal muscle changes. Aims of the present study were to investigate the progression and sequence of histopathologic changes caused by chronic IIH in rat peripheral nerves and skeletal muscle, and whether such changes were reversible...
August 16, 2017: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28812043/swallowing-markers-in-spinal-and-bulbar-muscular-atrophy
#2
Haruhiko Banno, Masahisa Katsuno, Keisuke Suzuki, Seiya Tanaka, Noriaki Suga, Atsushi Hashizume, Tomoo Mano, Amane Araki, Hirohisa Watanabe, Yasushi Fujimoto, Masahiko Yamamoto, Gen Sobue
OBJECTIVE: We examined the characteristics of dysphagia in spinal and bulbar muscular atrophy, a hereditary neuromuscular disease causing weakness of limb, facial, and oropharyngeal muscles via a videofluoroscopic swallowing study, and investigated the plausibility of using these outcome measures for quantitative analysis. METHODS: A videofluoroscopic swallowing study was performed on 111 consecutive patients with genetically confirmed spinal and bulbar muscular atrophy and 53 age- and sex-matched healthy controls...
August 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28811488/deregulation-of-zpr1-causes-respiratory-failure-in-spinal-muscular-atrophy
#3
Naresh K Genabai, Annapoorna Kannan, Saif Ahmad, Xiaoting Jiang, Kanchan Bhatia, Laxman Gangwani
Spinal muscular atrophy (SMA) is caused by the low levels of survival motor neuron (SMN) protein and is characterized by motor neuron degeneration and muscle atrophy. Respiratory failure causes death in SMA but the underlying molecular mechanism is unknown. The zinc finger protein ZPR1 interacts with SMN. ZPR1 is down regulated in SMA patients. We report that ZPR1 functions downstream of SMN to regulate HoxA5 levels in phrenic motor neurons that control respiration. Spatiotemporal inactivation of Zpr1 gene in motor neurons down-regulates HoxA5 and causes defects in the function of phrenic motor neurons that results in respiratory failure and perinatal lethality in mice...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28809518/mir-542-promotes-mitochondrial-dysfunction-and-smad-activity-and-is-raised-in-icu-acquired-weakness
#4
Roser Farre Garros, Richard Paul, Martin Connolly, Amy Lewis, Benjamin E Garfield, S Amanda Natanek, Susannah Bloch, Vincent Mouly, Mark J Griffiths, Michael I Polkey, Paul R Kemp
RATIONALE: Loss of skeletal muscle mass and function is a common consequence of critical illness and a range of chronic diseases but the mechanisms by which this occurs are unclear. OBJECTIVES: We aimed to identify miRNAs that were increased in the quadriceps of patients with muscle wasting and to determine the molecular pathways by which they contributed to muscle dysfunction. METHODS: miR-542-3p/-5p were quantified in the quadriceps of patients with COPD and intensive care unit acquired weakness (ICUAW)...
August 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28807824/flavan-3-ol-delays-the-progression-of-disuse-atrophy-induced-by-hindlimb-suspension-in-mice
#5
Mao Ito, Naoto Kudo, Yuji Miyake, Tatsuya Imai, Tomoki Unno, Yoko Yamashita, Yoshihisa Hirota, Hitoshi Ashida, Naomi Osakabe
Periods of skeletal muscle disuse, for example due to a sedentary lifestyle or bed rest, are associated with aging and can lead to muscle atrophy. We previously found that the flavan 3-ol fraction derived from cocoa (FL) enhanced energy expenditure with metabolic changes in skeletal muscle. In the present study, we examined the effect of FL on disuse muscle atrophy induced by hindlimb suspension in mice. Male C57BL/6J mice were assigned to four groups as follows: unsuspended-vehicle, unsuspended-FL, suspended-vehicle, and suspended-FL...
August 11, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28807072/clavicle-fracture-with-osteomyelitis-after-neck-dissection-and-post-operative-radiotherapy-case-report
#6
R Shodo, Y Sato, H Ota, A Horii
BACKGROUND: Non-traumatic bone fractures in cancer patients are usually pathological fractures due to bone metastases. In head and neck cancer patients, clavicle stress fractures may occur as a result of atrophy of the trapezius muscle after neck dissection in which the accessory nerve becomes structurally or functionally damaged. CASE REPORT: A 71-year-old man underwent modified radical neck dissection with accessory nerve preservation and post-operative radiotherapy for submandibular lymph node metastases of tongue cancer...
August 15, 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28804760/autopsy-case-of-the-c12orf65-mutation-in-a-patient-with-signs-of-mitochondrial-dysfunction
#7
Hideaki Nishihara, Masatoshi Omoto, Masaki Takao, Yujiro Higuchi, Michiaki Koga, Motoharu Kawai, Hiroo Kawano, Eiji Ikeda, Hiroshi Takashima, Takashi Kanda
OBJECTIVE: To describe the autopsy case of a patient with a homozygous 2-base deletion, c171_172delGA (p.N58fs), in the C12orf65 gene. METHODS: We described the clinical history, neuroimaging data, neuropathology, and genetic analysis of the patients with C12orf65 mutations. RESULTS: The patient was a Japanese woman with a history of delayed psychomotor development, primary amenorrhea, and gait disturbance in her 20s. She was hospitalized because of respiratory failure at the age of 60...
August 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28804634/muscle-mri-findings-in-a-one-year-old-girl-with-merosin-deficient-congenital-muscular-dystrophy-type-1a-due-to-lama2-mutation-a-case-report
#8
Yingyin Liang, Guidian Li, Songlin Chen, Rongxing He, Xiangxue Zhou, Yingming Chen, Xue Xu, Ronglan Zhu, Cheng Zhang
The objective of the present study was to characterize the muscle magnetic resonance imaging (MRI) features of a 1-year-old girl with merosin-deficient congenital muscular dystrophy type 1A (MDC1A). Beginning as an infant, this patient exhibited severe hypotonia and proximal weakness, as well as delays in developmental milestones. Her serum creatine kinase levels at 3 months, 8 months and 1 year were 2,959, 1,621 and 1,659 U/l, respectively. Brain MRI indicated symmetric, mild T1WI low, mild T2WI and FLAIR high radial patterns in the white matter of the Cornu posterius of the ventricular lateral...
August 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28804474/a-longitudinal-electromyography-study-of-complex-movements-in-poststroke-therapy-1-heterogeneous-changes-despite-consistent-improvements-in-clinical-assessments
#9
Negin Hesam-Shariati, Terry Trinh, Angelica G Thompson-Butel, Christine T Shiner, Penelope A McNulty
Poststroke weakness on the more-affected side may arise from reduced corticospinal drive, disuse muscle atrophy, spasticity, and abnormal coordination. This study investigated changes in muscle activation patterns to understand therapy-induced improvements in motor-function in chronic stroke compared to clinical assessments and to identify the effect of motor-function level on muscle activation changes. Electromyography (EMG) was recorded from five upper limb muscles on the more-affected side of 24 patients during early and late therapy sessions of an intensive 14-day program of Wii-based Movement Therapy (WMT) and for a subset of 13 patients at 6-month follow-up...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28803135/effects-of-aging-on-basement-membrane-of-the-soleus-muscle-during-recovery-following-disuse-atrophy-in-rats
#10
Yuji Kanazawa, Keisuke Ikegami, Mitsugu Sujino, Satoshi Koinuma, Mamoru Nagano, Yuki Oi, Tomoya Onishi, Shinichi Sugiyo, Isao Takeda, Hiroshi Kaji, Yasufumi Shigeyoshi
Aging is known to lead to the impaired recovery of muscle after disuse as well as the increased susceptibility of the muscle to damage. Here, we show that, in the older rats, reloading after disuse atrophy, causes the damage of the muscle fibers and the basement membrane (BM) that structurally support the muscle fibers. Male Wistar rats of 3-(young) and 20-(older) months of age were subjected to hindlimb-unloading for 2weeks followed by reloading for a week. In the older rats, the soleus muscles showed necrosis and central nuclei fiber indicating the regeneration of muscle fibers...
August 9, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28801086/slowly-progressive-leukodystrophy-in-an-adolescent-male-with-phosphoglycerate-kinase-deficiency
#11
Shimpei Baba, Ayumi Kobayashi, Haruna Yokoyama, Kengo Moriyama, Ayako Kashimada, Jun Oyama, Ayako Owada, Shoichi Oyama, Tomohiro Morio, Masatoshi Takagi
We report the case of an 18-year-old man with a phosphoglycerate kinase (PGK) deficiency who had slowly progressive leukodystrophy during adolescence. The patient had a history of severe neonatal jaundice, hemolytic crisis with rhabdomyolysis triggered by febrile viral infections, dysarthria, and intellectual disability during early childhood. Clumsiness in walking and writing became obvious at ∼10years of age. Evaluations performed by us on the 18-year-old patient confirmed the presence of pyramidal tract signs, increased muscle tone, and generalized dystonia...
August 8, 2017: Brain & Development
https://www.readbyqxmd.com/read/28800433/global-gene-expression-analysis-of-the-muscle-tissues-of-medaka-acclimated-to-low-and-high-environmental-temperatures
#12
Daisuke Ikeda, Hiroki Koyama, Nanami Mizusawa, Nobuhiro Kan-No, Engkong Tan, Shuichi Asakawa, Shugo Watabe
Medaka (Oryzias latipes) is a temperate eurythermal fish that is able to survive over a wide range of water temperatures ranging from near zero to over 30°C throughout the year; it maintains its normal physiological and biochemical processes through temperature acclimation. To determine the mechanisms involved in temperature acclimation of fish, the fast skeletal muscle tissues of medaka underwent global gene expression analysis using next-generation sequencing. Ten individuals were placed into two aquariums at 24°C...
August 2, 2017: Comparative Biochemistry and Physiology. Part D, Genomics & Proteomics
https://www.readbyqxmd.com/read/28800153/increased-autolysis-of-%C3%AE-calpain-in-skeletal-muscles-of-chronic-alcohol-fed-rats
#13
Yulia V Gritsyna, Nikolay N Salmov, Alexander G Bobylev, Anna D Ulanova, Nikolay I Kukushkin, Zoya A Podlubnaya, Ivan M Vikhlyantsev
BACKGROUND: Proteolysis can proceed via several distinct pathways such as the lysosomal, calcium-dependent, and ubiquitin (Ub)-proteasome-dependent pathways. Calpains are the main proteases that cleave a large variety of proteins, including the giant sarcomeric proteins titin and nebulin. Chronic ethanol feeding for 6 weeks did not affect the activities of μ-calpain and m-calpain in the m. gastrocnemius. In our research, changes in μ-calpain activity were studied in the m. gastrocnemius and m...
August 11, 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/28799578/nusinersen-antisense-oligonucleotide-to-increase-smn-protein-production-in-spinal-muscular-atrophy
#14
D M Paton
Patients with spinal muscular atrophy (SMA) have an autosomal recessive disease that limits their ability to produce survival motor neuron (SMN) protein in the CNS resulting in progressive wasting of voluntary muscles. Detailed studies over several years have demonstrated that phosphorothioate and 2'-O-methoxyethyl- modified antisense oligonucleotides (ASOs) targeting the ISS-N1 site increase SMN2 exon 7 inclusion, thus increasing levels of SMN protein in a dose- and time-dependent manner in liver, kidney and skeletal muscle, and CNS tissues only when administered intrathecally...
June 2017: Drugs of Today
https://www.readbyqxmd.com/read/28799033/isolated-atrophy-of-the-abductor-digiti-quinti-in-patients-with-rheumatoid-arthritis
#15
Ricardo Andrade Fernandes de Mello, Ronaldo Garcia Rondina, Valéria Valim, Stéphano Santos Belisario, Rafael Burgomeister Lourenço, Elton Francisco Batista, Ruben Horst Duque
OBJECTIVE: We aim to discuss the association of isolated atrophy of the abductor digiti quinti muscle in patients with rheumatoid arthritis as well as review the anatomy and imaging findings of this condition on MRI. MATERIALS AND METHODS: A consecutive series of 55 patients diagnosed with rheumatoid arthritis according to the 2010 ACR/EULAR classification criteria were recruited. MRI of the clinically dominant feet was performed using a 1.5-T scanner. RESULTS: The study population was predominantly female (94...
August 10, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28798877/previously-undiagnosed-spinal-and-bulbar-muscular-atrophy-as-a-cause-of-airway-obstruction-after-robot-assisted-laparoscopic-prostatectomy
#16
Miyuki Niki, Taihei Tachikawa, Yuka Sano, Hiroki Miyawaki, Aisa Matoi, Yukari Okano, Nobutaka Kariya, Tsuneo Tatara, Munetaka Hirose
BACKGROUND: Preoperative vocal cord paralysis is a risk factor for postoperative respiratory distress following extubation after general anesthesia. We present an unusual case where a geriatric patient developed airway obstruction after robot-assisted laparoscopic prostatectomy. CASE PRESENTATION: A 67-year-old male, who had suffered from left vocal cord paralysis of unknown etiology, was scheduled for robot-assisted laparoscopic prostatectomy (RALP). General anesthesia was performed without any problems...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28798690/risk-of-myopathy-in-patients-in-therapy-with-statins-identification-of-biological-markers-in-a-pilot-study
#17
Giulia M Camerino, Olimpia Musumeci, Elena Conte, Kejla Musaraj, Adriano Fonzino, Emanuele Barca, Marco Marino, Carmelo Rodolico, Domenico Tricarico, Claudia Camerino, Maria R Carratù, Jean-François Desaphy, Annamaria De Luca, Antonio Toscano, Sabata Pierno
Statin therapy may induce skeletal muscle damage ranging from myalgia to severe rhabdomyolysis. Our previous preclinical studies showed that statin treatment in rats involves the reduction of skeletal muscle ClC-1 chloride channel expression and related chloride conductance (gCl). An increase of the activity of protein kinase C theta (PKC theta) isoform, able to inactivate ClC-1, may contribute to destabilize sarcolemma excitability. These effects can be detrimental for muscle function leading to drug-induced myopathy...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28796007/mri-scoring-methods-used-in-evaluation-of-muscle-involvement-in-patients-with-idiopathic-inflammatory-myopathies
#18
Kateřina Kubínová, Heřman Mann, Jiří Vencovský
PURPOSE OF REVIEW: MRI is a promising imaging method commonly used to assess muscle involvement in patients with idiopathic inflammatory myopathies (IIM). MRI enables evaluation of both activity and damage and is therefore an ideal noninvasive diagnostic and monitoring tool. Despite its widespread use, there is no universally accepted method for scoring and reporting of MRI findings. The aim of this review is to provide an overview of systems used in the evaluation of MR images in patients with IIM...
August 8, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28795971/altered-satellite-cell-dynamics-accompany-skeletal-muscle-atrophy-during-chronic-illness-disuse-and-aging
#19
Colleen F McKenna, Christopher S Fry
PURPOSE OF REVIEW: This review explores recent research investigating the contribution of satellite cells (skeletal muscle stem cells) during muscle fiber atrophy as seen in periods of disuse, illness, and aging. RECENT FINDINGS: Studies indicate reduced satellite cell activity and density in a variety of acute and chronic conditions characterized by robust muscle wasting. The direct contribution of satellite cells to unloading/denervation and chronic illness-induced atrophy remains controversial...
August 8, 2017: Current Opinion in Clinical Nutrition and Metabolic Care
https://www.readbyqxmd.com/read/28794355/characteristic-posterior-dominant-lower-limb-muscle-involvement-in-limb-girdle-muscular-dystrophy-due-to-a-dnajb6-phe93leu-mutation
#20
Yuta Kojima, Yu-Ichi Noto, Daiki Takewaki, Naoki Tokuda, Kensuke Shiga, Ai Hamano, Ikuko Mizuta, Manabu Muranishi, Takashi Kasai, Masanori Nakagawa, Toshiki Mizuno
A 41-year-old man presented with gradually progressing proximal-dominant lower limb atrophy and weakness. His brother, mother and maternal aunt had the same symptoms. A physical examination and muscle imaging (CT and ultrasound) showed selective muscle involvement of the bilateral paraspinal, gluteus and posterior groups of lower limb muscles. Based on the characteristic muscle involvement pattern, the clinical findings and the muscle biopsy results, we made a straightforward diagnosis of limb-girdle muscular dystrophy (LGMD) due to a DNAJB6 Phe93Leu mutation based on a targeted gene analysis...
August 10, 2017: Internal Medicine
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