Results 81 to 90 of about 4,634 (224)

Proteomic Profiling of Myofiber Repair Annexins and Their Role in Duchenne Muscular Dystrophy

PROTEOMICS, EarlyView.
ABSTRACT Myofiber regeneration and membrane repair play crucial roles in maintaining the continuous physiological functioning of the neuromuscular system. A swift and efficient repair mechanism enables the rapid restoration of sarcolemmal integrity following cellular impairment in damaged skeletal muscles.
Paul Dowling, Dounia Bouragba, Elisa Negroni, Capucine Trollet, Margit Zweyer, Dieter Swandulla, Kay Ohlendieck   +6 more
wiley   +1 more source

Dysferlin-derived peptides. [PDF]

, 2012
Dysferlin-derived peptides.
Peter T. Daniel (118346), Andreas Marg (118333), Severine Kunz (118335), Verena Schoewel (118330), Ute Zacharias (118344), Simone Spuler (118350), Tim Overkamp (118338), Romy Siegert Carrazedo (118341)   +7 more
core   +1 more source

Correction: Dysferlin-deficiency has greater impact on function of slow muscles, compared with fast, in aged BLAJ mice

PLoS ONE, 2023
[This corrects the article DOI: 10.1371/journal.pone.0214908.].
E. Lloyd, Hongyang Xu, R. Murphy, M. Grounds, G. Pinniger   +4 more
semanticscholar   +1 more source

Diagnostic utility of inflammatory markers in formalin‐fixed and paraffin‐embedded muscle biopsies for idiopathic inflammatory myopathies

Histopathology, EarlyView.
Formalin‐fixed, paraffin‐embedded (FFPE) muscle tissue supports robust immunohistochemical detection of MHC II, MxA, and p62 with performance comparable to frozen sections. This approach reliably identifies the pathological signatures of inclusion body myositis, dermatomyositis, immune‐mediated necrotizing myopathy, and overlap myositis, enhancing the ...
Chinnawut Suriyonplengsaeng, Jariya Waisayarat   +1 more
wiley   +1 more source

Characterization of FER1L5, a novel dysferlin myoferlin related protein [PDF]

, 2009
The ferlins are mammalian homologues of the C-elegans sperm vesicle fusion protein FER-1 characterised by multiple C2 domains and a C-terminal anchor.
Ramachandran, Usha Kalyani
core  

Pharmacotherapeutic Approaches to Treatment of Muscular Dystrophies

Biomolecules, 2023
Muscular dystrophies are a heterogeneous group of genetic muscle-wasting disorders that are subdivided based on the region of the body impacted by muscle weakness as well as the functional activity of the underlying genetic mutations. A common feature of
Alan Rawls, Bridget K. Diviak, Cameron I. Smith, Grant W. Severson, Sofia A. Acosta, Jeanne Wilson-Rawls   +5 more
doaj   +1 more source

Cholesterol absorption blocker ezetimibe prevents muscle wasting in severe dysferlin‐deficient and mdx mice

Journal of Cachexia, Sarcopenia and Muscle, 2021
Muscular dystrophy (MD) causes muscle wasting and is often lethal in patients due to a lack of proven therapies. In contrast, mouse models of MD are notoriously mild.
Z. White, M. Théret, Nadia Milad, Lin Wei Tung, William Wei-Han Chen, M. Sirois, F. Rossi, P. Bernatchez   +7 more
semanticscholar   +1 more source

Targeting progressive multiple sclerosis: Toward mechanism‐informed precision medicine

Journal of Internal Medicine, EarlyView.
Abstract Multiple sclerosis has undergone a therapeutic revolution over the past three decades. Randomized clinical trials and real‐world data demonstrate that modern disease‐modifying therapies substantially reduce relapse rates and acute inflammatory activity detected by magnetic resonance imaging (MRI).
Fredrik Piehl, Gonçalo Castelo‐Branco, Maja Jagodic, Tomas Olsson   +3 more
wiley   +1 more source

Dysferlin-peptides redirect mutant dysferlin to the sarcolemma in primary human myotubes. [PDF]

, 2012
Primary human myotubes carrying dysferlin missense mutations were treated with the TAT-labeled dysferlin-peptides. Dysferlin was detected by anti-dysferlin ab. Nuclei are stained with Hoechst. Missense mutated dysferlin aggregates within the myotubes (A,
Peter T. Daniel (118346), Andreas Marg (118333), Severine Kunz (118335), Verena Schoewel (118330), Ute Zacharias (118344), Simone Spuler (118350), Tim Overkamp (118338), Romy Siegert Carrazedo (118341)   +7 more
core   +1 more source

Clinical, Neurophysiological, Radiological, Pathological, and Genetic Features of Dysferlinopathy in Saudi Arabia

Frontiers in Neuroscience, 2022
BackgroundTo characterize the phenotypic, neurophysiological, radiological, pathological, and genetic profile of 33 Saudi Arabian families with dysferlinopathy.MethodsA descriptive observational study was done on a cohort of 112 Saudi Arabian families ...
Norah Alharbi, Rawan Matar, Edward Cupler, Hindi Al-Hindi, Hatem Murad, Iftteah Alhomud, Dorota Monies, Ali Alshehri, Mossaed Alyahya, Brian Meyer, Saeed Bohlega   +10 more
doaj   +1 more source