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Muscular chains

Kinga I Gawlik, Vahid M Harandi, Rachel Y Cheong, Åsa Petersén, Madeleine Durbeej
Muscular dystrophies, including laminin α2 chain-deficient muscular dystrophy (LAMA2-CMD), are associated with immense personal, social and economic burdens. Thus, effective treatments are urgently needed. LAMA2-CMD is either a severe, early-onset condition with complete laminin α2 chain-deficiency or a milder, late-onset form with partial laminin α2 chain-deficiency. Mouse models dy3K /dy3K and dy2J /dy2J , respectively, recapitulate these two forms of LAMA2-CMD very well. We have previously demonstrated that laminin α1 chain significantly reduces muscular dystrophy in laminin α2 chain-deficient dy3K /dy3K mice...
March 12, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
Val A Fajardo, John S Mikhaeil, Cameron F Leveille, A Russell Tupling, Paul J LeBlanc
BACKGROUND: An increase in phosphatidylcholine:phosphatidylethanolamine (PC:PE) and a decrease in fatty acyl chain length, monounsaturated:polyunsaturated (MUFA:PUFA) fatty acyl ratio reduces SERCA activity in liposomes and in mouse models of obesity and muscular dystrophy. We have previously shown that maximal SERCA activity is significantly reduced in mechanically overloaded (OVL) plantaris, however, whether changes in PC:PE ratio or fatty acyl composition may contribute to the alterations in maximal SERCA activity remain unknown...
March 13, 2018: Lipids in Health and Disease
Sara C Atehortúa, Luz H Lugo, Mateo Ceballos, Esteban Orozco, Paula A Castro, Juan C Arango, Heidi E Mateus
OBJECTIVES: To determine the cost-effectiveness ratio of different courses of action for the diagnosis of Duchenne or Becker muscular dystrophy in Colombia. METHODS: The cost-effectiveness analysis was performed from the Colombian health system perspective. Decision trees were constructed, and different courses of action were compared considering the following tests: immunohistochemistry (IHC), Western blot (WB), multiplex polymerase chain reaction, multiplex ligation-dependent probe amplification (MLPA), and the complete sequencing of the dystrophin gene...
March 9, 2018: Value in Health Regional Issues
Clemente Lauretti, Francesca Cordella, Anna Lisa Ciancio, Emilio Trigili, Jose Maria Catalan, Francisco Javier Badesa, Simona Crea, Silvio Marcello Pagliara, Silvia Sterzi, Nicola Vitiello, Nicolas Garcia Aracil, Loredana Zollo
The reference joint position of upper-limb exoskeletons is typically obtained by means of Cartesian motion planners and inverse kinematics algorithms with the inverse Jacobian; this approach allows exploiting the available Degrees of Freedom (i.e. DoFs) of the robot kinematic chain to achieve the desired end-effector pose; however, if used to operate non-redundant exoskeletons, it does not ensure that anthropomorphic criteria are satisfied in the whole human-robot workspace. This paper proposes a motion planning system, based on Learning by Demonstration, for upper-limb exoskeletons that allow successfully assisting patients during Activities of Daily Living (ADLs) in unstructured environment, while ensuring that anthropomorphic criteria are satisfied in the whole human-robot workspace...
2018: Frontiers in Neurorobotics
Dariusz Czaprowski, Łukasz Stoliński, Marcin Tyrakowski, Mateusz Kozinoga, Tomasz Kotwicki
Background: The physiological sagittal spinal curvature represents a typical feature of good body posture in the sagittal plane. The cervical and the lumbar spine are curved anteriorly (lordosis), while the thoracic segment is curved posteriorly (kyphosis). The pelvis is inclined anteriorly, and the lower limbs' joints remain in a neutral position. However, there are many deviations from the optimal body alignment.The aim of this paper is to present the most common types of non-structural misalignments of the body posture in the sagittal plane...
2018: Scoliosis and Spinal Disorders
Chamara Arachchighe Lahiru Weerasinghe, Bich-Hong Thi Bui, Thu Thi Vu, Hong-Loan Thi Nguyen, Bao-Khanh Phung, Van-Minh Nguyen, Van-Anh Pham, Vu-Hung Cao, Tuan-Nghia Phan
Leigh syndrome is a rare inherited, heterogeneous and progressive neurometabolic disorder that is mainly caused by specific mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA). The present study reported a case of childhood Leigh syndrome with a point mutation at bp 8,993 in the mitochondrial ATPase6 gene. A 21‑month‑old male child had developed epilepsy, muscular weakness and vomiting, which was accompanied by high fever. Magnetic resonance imaging indicated typical characteristics of Leigh syndrome, including a symmetric abnormal signal in the dorsal medulla oblongata and Sylvian fissure enlargement in association with an abnormal signal in the periventricular white matter and in the putamina and caudate heads...
March 1, 2018: Molecular Medicine Reports
Carolina Becerril-Esquivel, Katia Peñuelas-Urquides, Erik Blancas-Sánchez, Pablo Zapata-Benavides, Beatriz Silva-Ramírez, Arturo Chávez-Reyes, Fabiola Castorena-Torres, Bulmaro Cisneros, Mario Bermúdez de León
The smallest product of the Duchenne muscular dystrophy gene, dystrophin (Dp)71, is ubiquitously expressed in nonmuscle tissues. We previously showed that Dp71 expression in hepatic cells is modulated in part by stimulating factor 1 (Sp1), stimulating protein 3 (Sp3), and yin yang 1 (YY1) transcription factors, and that the polyaromatic hydrocarbon, β-naphthoflavone (β‑NF), downregulates Dp71 expression. The aim of the present study was to determine whether β‑NF represses Dp71 expression by altering mRNA stability or its promoter activity...
February 21, 2018: Molecular Medicine Reports
Justin R Fallon, Elizabeth M McNally
The extracellular matrix (ECM) plays key roles in normal and diseased skeletal and cardiac muscle. In healthy muscle the ECM is essential for transmitting contractile force, maintaining myofiber integrity and orchestrating cellular signaling. Duchenne Muscular Dystrophy (DMD) is caused by loss of dystrophin, a cytosolic protein that anchors a transmembrane complex and serves as a vital link between the actin cytoskeleton and the basal lamina. Loss of dystrophin leads to membrane fragility and impaired signaling, resulting in myofiber death and cycles of inflammation and regeneration...
February 23, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
Lluís Sánchez, Elsa Beltrán, Alberta de Stefani, Ling T Guo, Anita Shea, G Diane Shelton, Luisa De Risio, Louise M Burmeister
Four full-sibling intact male Miniature Poodles were evaluated at 4-19 months of age. One was clinically normal and three were affected. All affected dogs were reluctant to exercise and had generalised muscle atrophy, a stiff gait and a markedly elevated serum creatine kinase activity. Two affected dogs also showed poor development, learning difficulties and episodes of abnormal behaviour. In these two dogs, investigations into forebrain structural and metabolic diseases were unremarkable; electromyography demonstrated fibrillation potentials and complex repetitive discharges in the infraspinatus, supraspinatus and epaxial muscles...
2018: PloS One
Daria Domańska-Senderowska, Zbigniew Jastrzębski, Justyna Kiszałkiewicz, Michał Brzeziański, Dorota Pastuszak-Lewandoska, Łukasz Radzimińki, Ewa Brzeziańska-Lasota, Anna Jegier
Recently studies have shown that, depending on the type of training and its duration, the expression levels of selected circulating myomiRNAs (c-miR-27a,b, c-miR-29a,b,c, c-miR-133a) differ and correlate with the physiological indicators of adaptation to physical activity. To analyse the expression of selected classes of miRNAs in soccer players during different periods of their training cycle. The study involved 22 soccer players aged 17-18 years. The multi-stage 20-m shuttle run test was used to estimate VO2 max among the soccer players...
December 2017: Biology of Sport
Gautam Pareek, Ruth E Thomas, Leo J Pallanck
The progressive accumulation of dysfunctional mitochondria is implicated in aging and in common diseases of the elderly. To oppose this occurrence, organisms employ a variety of strategies, including the selective degradation of oxidatively damaged and misfolded mitochondrial proteins. Genetic studies in yeast indicate that the ATPase Associated with diverse cellular Activities (AAA+ ) family of mitochondrial proteases account for a substantial fraction of this protein degradation, but their metazoan counterparts have been little studied, despite the fact that mutations in the genes encoding these proteases cause a variety of human diseases...
February 21, 2018: Cell Death & Disease
Dorota Piekutowska-Abramczuk, Zahra Assouline, Lavinija Mataković, René G Feichtinger, Eliška Koňařiková, Elżbieta Jurkiewicz, Piotr Stawiński, Mirjana Gusic, Andreas Koller, Agnieszka Pollak, Piotr Gasperowicz, Joanna Trubicka, Elżbieta Ciara, Katarzyna Iwanicka-Pronicka, Dariusz Rokicki, Sylvain Hanein, Saskia B Wortmann, Wolfgang Sperl, Agnès Rötig, Holger Prokisch, Ewa Pronicka, Rafał Płoski, Giulia Barcia, Johannes A Mayr
Respiratory chain complex I deficiency is the most frequently identified biochemical defect in childhood mitochondrial diseases. Clinical symptoms range from fatal infantile lactic acidosis to Leigh syndrome and other encephalomyopathies or cardiomyopathies. To date, disease-causing variants in genes coding for 27 complex I subunits, including 7 mitochondrial DNA genes, and in 11 genes encoding complex I assembly factors have been reported. Here, we describe rare biallelic variants in NDUFB8 encoding a complex I accessory subunit revealed by whole-exome sequencing in two individuals from two families...
March 1, 2018: American Journal of Human Genetics
Joseph E Rupert, J Ethan Joll, Wiaam Y Elkhatib, Jason M Organ
The musculoskeletal system is remarkably plastic during growth. The purpose of this study was to examine the muscular plasticity in functional and structural properties in a model known to result in significant developmental plasticity of the postcranial skeleton. Fifteen weanling C57BL/6 mice were raised to 16 weeks of age in one of two enclosures: a climbing enclosure that simulates a fine branch arboreal habitat and is traversed by steel wires crossing at 45° relative to horizontal at multiple intersections, and a control enclosure that resembles a parking deck with no wires but the same volume of habitable space...
March 2018: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
Motoi Kanagawa, Tatsushi Toda
Glycosylation is a crucial posttranslational modification that is involved in numerous biological events. Therefore, abnormal glycosylation can impair the functions of glycoproteins or glycolipids and is occasionally associated with cell dysfunction and human diseases. For example, aberrant glycosylation of dystroglycan, a cellular receptor for matrix and synaptic proteins, is associated with muscular dystrophy and lissencephaly. Dystroglycan sugar chains are required for high-affinity binding to ligand proteins, and thus disruption of dystroglycan-ligand linkages underlies disease conditions...
January 31, 2018: Journal of Biochemistry
Jaime Felipe Guerrero-Garzón, Melisa Bénard-Valle, Rita Restano-Cassulini, Fernando Zamudio, Gerardo Corzo, Alejandro Alagón, Alejandro Olvera-Rodríguez
The three-finger toxins (3FTxs) represent an extremely diverse protein family in elapid venoms, where the short chain α-neurotoxins are the most relevant toxin group from the clinical point of view. Essentially, the 3FTxs variability and the low proportions of α-neurotoxins in the venoms of North American coral snakes make it difficult to obtain effective elapid antivenoms against the envenomation symptoms caused mainly by these α-neurotoxins. In this work, thirty 3FTx transcript sequences were obtained from the venom glands of four coral snake species from Mexico (M...
January 29, 2018: Biochimie
Birgit Friedmann-Bette, Francesca Profit, Thomas Gwechenberger, Nadine Weiberg, Mario Parstorfer, Marc-Andre Weber, Nikolaus Streich, Alexander Barié
PURPOSE: Protracted quadriceps muscle atrophy is observed after anterior cruciate ligament reconstruction (ACL-R). The aim of this study was to assess if quadriceps strength training with eccentric overload (CON/ECC) is more efficient to induce muscle regeneration after ACL-R than conventional concentric/eccentric (CON/ECC) strength training. METHODS: Biopsies from the vastus lateralis muscle were obtained from 37 recreational athletes after 12 weeks of regular rehabilitation following ACL-R and again after 12 weeks with twice a week either conventional CON/ECC (n = 16) or CON/ECC (n = 21) one-legged supervised leg-press training...
January 31, 2018: Medicine and Science in Sports and Exercise
Juan Chen, Jingyang Wang, Jiyan Zhang, Chuanqiang Pu
Idiopathic inflammatory myopathies are a group of rare muscular diseases that are characterized by acute, subacute or chronic proximal and symmetric muscle weakness, muscle fiber necrosis and infiltration of inflammatory cells, particularly activated CD8+ cytotoxic T cells and phagocytes. 3-n-butylphthalide (NBP) protects mitochondria and reduces the inflammatory response in multiple disease models. In myositis, it has remained elusive whether NBP can protect muscle cells from muscle fiber injury. Experimental autoimmune myositis (EAM) was induced in a total of 40 guinea pigs by myosin immunization...
January 2018: Experimental and Therapeutic Medicine
Lena Boberg, Ferenc L M Szekeres, Anders Arner
This study aims to improve the classification of smooth muscle types to better understand their normal and pathological functional phenotypes. Four different smooth muscle tissues (aorta, muscular arteries, intestine, urinary bladder) with a 5-fold difference in maximal shortening velocity were obtained from mice and classified according to expression of the inserted myosin heavy chain (SMHC-B). Western blotting and quantitative PCR analyses were used to determine 15 metabolic and 8 cell signaling key components in each tissue...
January 29, 2018: Pflügers Archiv: European Journal of Physiology
Yifeng Jiang, Zhen Du, Long Chen
Numerous factors, including trauma, tumors and myophagism, may lead to tongue defects, which are mostly repaired via muscular flaps. However, these methods cannot restore the muscular function and gustation function of the tongue. Intensive research on tongue development may offer useful clues for tongue regeneration based on tissue engineering or stem cell therapy. In the present study, staining results revealed that tongue muscle fibers became larger, mature and stronger, and the foliate and fungiform papillae also became mature from newborn to adult C57BL/6J genetic background mice...
January 2018: Experimental and Therapeutic Medicine
Woo-Seok Bang, Dong-Hyun Lee, Kyoung-Tae Kim, Dae-Chul Cho, Joo-Kyung Sung, In-Bo Han, Du-Hwan Kim, Brian K Kwon, Chi Heon Kim, Ki-Su Park, Man-Kyu Park, Sung-Young Seo, Ye-Jin Seo
BACKGROUND CONTEXT: Vitamin D deficiency (VDD) has been closely linked with skeletal muscle atrophy in many studies, but to date no study has focused on paraspinal muscle. PURPOSE: To verify paraspinal muscle changes according to serum vitamin D level. STUDY DESIGN: A cross-sectional study of patients who visited our hospital and an in vivo animal study METHODS: We measured serum vitamin D concentration in 91 elderly women and stratified them according to their vitamin D status in three groups: control, vitamin D insufficiency, and VDD, and obtained magnetic resonance imaging data of the lumbar spine and evaluated the quality and quantity of the paraspinal muscles...
January 16, 2018: Spine Journal: Official Journal of the North American Spine Society
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