1,305 results match your criteria agrin

The extracellular matrix protein agrin is essential for epicardial epithelial-to-mesenchymal transition during heart development.

Development 2021 May 10;148(9). Epub 2021 May 10.

Burdon-Sanderson Cardiac Science Centre, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3PT, UK.

During heart development, epicardial cells residing within the outer layer undergo epithelial-mesenchymal transition (EMT) and migrate into the underlying myocardium to support organ growth and morphogenesis. Disruption of epicardial EMT results in embryonic lethality, yet its regulation is poorly understood. Here, we report epicardial EMT within the mesothelial layer of the mouse embryonic heart at ultra-high resolution using scanning electron microscopy combined with immunofluorescence analyses. Read More

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Autoantibodies to cortactin and agrin in sera of patients with myasthenia gravis.

J Neuroimmunol 2021 Apr 30;356:577588. Epub 2021 Apr 30.

University Hospital Würzburg, Department of Neurology, Josef-Schneider-Str. 11, 97080 Würzburg, Germany. Electronic address:

Autoantibodies against agrin and cortactin have been described in patients with myasthenia gravis. To further validate and characterize these autoantibodies, sera and/or plasma exchange material of 135 patients with myasthenia gravis were screened for anti-agrin or anti-cortactin autoantibodies. Autoantibodies against cortactin were detected in three patients and two controls and could be confirmed by cell-based assays using cortactin-transfected human embryonic kidney cells in both controls and one patient, but were not detectable in follow-up sera of the three patients. Read More

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Heterologous fibrin biopolymer associated to a single suture stitch enables the return of neuromuscular junction to its mature pattern after peripheral nerve injury.

Injury 2021 Apr 17;52(4):731-737. Epub 2020 Oct 17.

Department of Structural and Functional Biology (Anatomy Sector), Institute of Biosciences, São Paulo State University (Unesp), Botucatu, São Paulo, Brazil. Electronic address:

Denervation leads to severe atrophy of neuromuscular junction (NMJ) structure including decrease of the expression of fundamental proteins. Up to now, conventional suture has been the gold standard method used to correct this injury. Fibrin sealant is one of the alternatives proposed to optimize this method. Read More

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Prediction of sarcopenia using a battery of circulating biomarkers.

Sci Rep 2021 Apr 21;11(1):8632. Epub 2021 Apr 21.

Department of Cardiology, Post Graduate Medical Institute, Hayatabad Medical Complex, Peshawar, Pakistan.

Loss of muscle mass and strength with aging, termed sarcopenia is accelerated in several comorbidities including chronic heart failure (CHF) and chronic obstructive pulmonary diseases (COPD). However, the effective circulating biomarkers to accurately diagnose and assess sarcopenia are not known. We recruited male healthy controls and patients with CHF and COPD (n = 81-87/group), aged 55-74 years. Read More

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Neuromuscular junction instability and altered intracellular calcium handling as early determinants of force loss during unloading in humans.

J Physiol 2021 Apr 21. Epub 2021 Apr 21.

Department of Biomedical Sciences, University of Padova, Padova, 35131, Italy.

Key Points: Few days of unloading are sufficient to induce a decline of skeletal muscle mass and function; notably, contractile force is lost at a faster rate than mass. The reasons behind this disproportionate loss of muscle force are still poorly understood. We provide strong evidence of two mechanisms only hypothesized until now for the rapid muscle force loss in only 10 days of bed rest. Read More

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Conservation and Innovation: Versatile Roles for LRP4 in Nervous System Development.

J Dev Biol 2021 Mar 14;9(1). Epub 2021 Mar 14.

Department of Neuroscience, Thomas Jefferson University, Philadelphia, PA 19107, USA.

As the nervous system develops, connections between neurons must form to enable efficient communication. This complex process of synaptic development requires the coordination of a series of intricate mechanisms between partner neurons to ensure pre- and postsynaptic differentiation. Many of these mechanisms employ transsynaptic signaling via essential secreted factors and cell surface receptors to promote each step of synaptic development. Read More

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Three-Dimensional Human Neural Stem Cell Models to Mimic Heparan Sulfate Proteoglycans and the Neural Niche.

Semin Thromb Hemost 2021 Apr 1;47(3):308-315. Epub 2021 Apr 1.

Genomics Research Centre, Stem Cell and Neurogenesis Group, Centre for Genomics and Personalised Health, School of Biomedical Science, Queensland University of Technology, Kelvin Grove, Queensland, Australia.

Heparan sulfate proteoglycans (HSPGs) are a diverse family of polysaccharides, consisting of a core protein with glycosaminoglycan (GAG) side chains attached. The heterogeneous GAG side-chain carbohydrates consist of repeating disaccharides, with each side chain possessing a specific sulfation pattern. It is the variable sulfation pattern that allows HSPGs to interact with numerous ligands including growth factors, cytokines, chemokines, morphogens, extracellular matrix (ECM) glycoproteins, collagens, enzymes, and lipases. Read More

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The epidemiology of myasthenia gravis.

J Med Life 2021 Jan-Mar;14(1):7-16

Neurology Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy.

Neuromuscular junction (NMJ) disorders include several dysfunctions that ultimately lead to muscle weakness. Myasthenia gravis (MG) is the most prevalent NMJ disorder with a highly polymorphic clinical presentation and many different faces. Being an autoimmune disease, MG correlates with the presence of detectable antibodies directed against the acetylcholine receptor, muscle-specific kinase, lipoprotein-related protein 4, agrin, titin, and ryanodine in the postsynaptic membrane at the NMJ. Read More

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Measuring and Monitoring Skeletal Muscle Mass after Stroke: A Review of Current Methods and Clinical Applications.

J Stroke Cerebrovasc Dis 2021 Jun 20;30(6):105736. Epub 2021 Mar 20.

Department of Rehabilitation Medicine, Okayama University Hospital, 2-5-1 shikata, Kitaku, Okayama 700-8558, Japan.

Objectives: Muscle mass at admission is important to survive stroke, and stroke-induced sarcopenia is a serious problem because of its poor prognosis. Muscle mass measurement and monitoring are essential for appropriate rehabilitation and nutrition management. However, few reviews are available about the muscle mass measurement and monitoring after stroke. Read More

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Sensing and Responding of Cardiomyocytes to Changes of Tissue Stiffness in the Diseased Heart.

Front Cell Dev Biol 2021 26;9:642840. Epub 2021 Feb 26.

Institute of Biochemistry and Biology, University of Potsdam, Potsdam, Germany.

Cardiomyocytes are permanently exposed to mechanical stimulation due to cardiac contractility. Passive myocardial stiffness is a crucial factor, which defines the physiological ventricular compliance and volume of diastolic filling with blood. Heart diseases often present with increased myocardial stiffness, for instance when fibrotic changes modify the composition of the cardiac extracellular matrix (ECM). Read More

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February 2021

Secreted Signaling Molecules at the Neuromuscular Junction in Physiology and Pathology.

Int J Mol Sci 2021 Feb 28;22(5). Epub 2021 Feb 28.

Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.

Signal transduction at the neuromuscular junction (NMJ) is affected in many human diseases, including congenital myasthenic syndromes (CMS), myasthenia gravis, Lambert-Eaton myasthenic syndrome, Isaacs' syndrome, Schwartz-Jampel syndrome, Fukuyama-type congenital muscular dystrophy, amyotrophic lateral sclerosis, and sarcopenia. The NMJ is a prototypic cholinergic synapse between the motor neuron and the skeletal muscle. Synaptogenesis of the NMJ has been extensively studied, which has also been extrapolated to further understand synapse formation in the central nervous system. Read More

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February 2021

What Is in the Neuromuscular Junction Literature?

J Clin Neuromuscul Dis 2021 Mar;22(3):147-154

Department of Neurology, University at Buffalo Jacobs School of Medicine and Biomedical Sciences/SUNY, Buffalo, NY.

Abstract: This update covers recommendations for myasthenia gravis (MG) in patients with coronavirus 2019 disease as well as reports of the clinical features of patients with MG and coronavirus 2019. Updated advisory committee recommendations for the use of thymectomy in generalized MG are also provided. Other MG topics include lipoprotein receptor-4 and agrin antibody associations, factors influencing conversion of ocular to generalized MG, the use of rituximab for more recent onset disease, immunoglobulins for maintenance therapy, and fatigue and depression. Read More

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The skeletome of the red coral Corallium rubrum indicates an independent evolution of biomineralization process in octocorals.

BMC Ecol Evol 2021 01 11;21(1). Epub 2021 Jan 11.

Centre Scientifique de Monaco, 8 Quai Antoine 1er, Monaco, MC, 98000, Monaco.

Background: The process of calcium carbonate biomineralization has arisen multiple times during metazoan evolution. In the phylum Cnidaria, biomineralization has mostly been studied in the subclass Hexacorallia (i.e. Read More

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January 2021

Molecular mechanisms of osteogenesis and antibacterial activity of Cu-bearing Ti alloy in a bone defect model with infection .

J Orthop Translat 2021 Mar 28;27:77-89. Epub 2020 Dec 28.

Department of Orthopaedics, Nanfang Hospital, Southern Medical University; Guangdong Provincial Key Laboratory of Bone and Cartilage Regenerative Medicine, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong, China.

Objective: The antibacterial activity of copper (Cu)-alloy biomaterials has shown a great potential in clinical application. Here, we evaluated the osteogenesis and antibacterial effects of Ti6Al4V-6.5wt%Cu alloy in an in vivo model of infected bone defects and determine their responsible proteins and pathways using proteomics. Read More

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Sixty days of head-down tilt bed rest with or without artificial gravity do not affect the neuromuscular secretome.

Exp Cell Res 2021 Feb 29;399(2):112463. Epub 2020 Dec 29.

Manchester Metropolitan University, Research Centre for Musculoskeletal Science & Sports Medicine, Faculty of Science and Engineering, John Dalton Building, Manchester, United Kingdom; Lithuanian Sports University, Institute of Sport Science and Innovations, Kaunas, Lithuania.

Artificial gravity is a potential countermeasure to attenuate effects of weightlessness during long-term spaceflight, including losses of muscle mass and function, possibly to some extent attributable to disturbed neuromuscular interaction. The 60-day AGBRESA bed-rest study was conducted with 24 participants (16 men, 8 women; 33 ± 9 years; 175 ± 9 cm; 74 ± 10 kg; 8 control group, 8 continuous (cAG) and 8 intermittent (iAG) centrifugation) to assess the impact of bed rest with or without daily 30-min continuous/intermittent centrifugation with 1G at the centre of mass. Fasting blood samples were collected before and on day 6, 20, 40 and 57 during 6° head-down tilt bed rest. Read More

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February 2021

The Neuromuscular Junction in Health and Disease: Molecular Mechanisms Governing Synaptic Formation and Homeostasis.

Front Mol Neurosci 2020 3;13:610964. Epub 2020 Dec 3.

Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom.

The neuromuscular junction (NMJ) is a highly specialized synapse between a motor neuron nerve terminal and its muscle fiber that are responsible for converting electrical impulses generated by the motor neuron into electrical activity in the muscle fibers. On arrival of the motor nerve action potential, calcium enters the presynaptic terminal, which leads to the release of the neurotransmitter acetylcholine (ACh). ACh crosses the synaptic gap and binds to ACh receptors (AChRs) tightly clustered on the surface of the muscle fiber; this leads to the endplate potential which initiates the muscle action potential that results in muscle contraction. Read More

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December 2020

Myasthenia Gravis: Autoantibody Specificities and Their Role in MG Management.

Front Neurol 2020 30;11:596981. Epub 2020 Nov 30.

Tzartos NeuroDiagnostics, Athens, Greece.

Myasthenia gravis (MG) is the most common autoimmune disorder affecting the neuromuscular junction, characterized by skeletal muscle weakness and fatigability. It is caused by autoantibodies targeting proteins of the neuromuscular junction; ~85% of MG patients have autoantibodies against the muscle acetylcholine receptor (AChR-MG), whereas about 5% of MG patients have autoantibodies against the muscle specific kinase (MuSK-MG). In the remaining about 10% of patients no autoantibodies can be found with the classical diagnostics for AChR and MuSK antibodies (seronegative MG, SN-MG). Read More

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November 2020

Pelvic floor muscle function recovery using biofabricated tissue constructs with neuromuscular junctions.

Acta Biomater 2021 02 13;121:237-249. Epub 2020 Dec 13.

Wake Forest Institute for Regenerative Medicine, Wake Forest School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1094, USA. Electronic address:

Damages in pelvic floor muscles often cause dysfunction of the entire pelvic urogenital system, which is clinically challenging. A bioengineered skeletal muscle construct that mimics structural and functional characteristics of native skeletal muscle could provide a therapeutic option to restore normal muscle function. However, most of the current bioengineered muscle constructs are unable to provide timely innervation necessary for successful grafting and functional recovery. Read More

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February 2021

Secretome Analysis of Inductive Signals for BM-MSC Transdifferentiation into Salivary Gland Progenitors.

Int J Mol Sci 2020 Nov 28;21(23). Epub 2020 Nov 28.

Oral and Maxillofacial Diagnostic Sciences, University of Florida College of Dentistry, Gainesville, FL 32610, USA.

Severe dry mouth in patients with Sjögren's Syndrome, or radiation therapy for patients with head and neck cancer, significantly compromises their oral health and quality of life. The current clinical management of xerostomia is limited to palliative care as there are no clinically-proven treatments available. Previously, our studies demonstrated that mouse bone marrow-derived mesenchymal stem cells (mMSCs) can differentiate into salivary progenitors when co-cultured with primary salivary epithelial cells. Read More

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November 2020

Agrin Yes-associated Protein Promotes the Proliferation of Epicardial Cells.

J Cardiovasc Pharmacol 2021 01;77(1):94-99

Department of Cardiology, the Second Affiliated Hospital of Chongqing Medical University, Chongqing, China.

Abstract: Embryonic epicardial cells make an important contribution to cardiac development. However, their proliferation mechanism is still unclear. Epicardial cells from E12. Read More

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January 2021

Agrin Involvement in Synaptogenesis Induced by Exercise in a Rat Model of Experimental Stroke.

Neurorehabil Neural Repair 2020 12 2;34(12):1124-1137. Epub 2020 Nov 2.

Yunnan University of Traditional Chinese Medicine, Kunming, China.

Background: Agrin is a proteoglycan that aggregates nicotinic acetylcholine receptors (AChRs) on neuromuscular junctions and takes part in synaptogenesis in the development of the central nervous system. However, its effects on neural repair and synaptogenesis after stroke are still unclear.

Objective: This study aimed to investigate the effects of agrin on neural repair and synaptogenesis after stroke and the effects of exercise on this process in vivo and in vitro. Read More

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December 2020

The lncRNA H19 alleviates muscular dystrophy by stabilizing dystrophin.

Nat Cell Biol 2020 11 26;22(11):1332-1345. Epub 2020 Oct 26.

Department of Molecular and Cellular Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Dystrophin proteomic regulation in muscular dystrophies (MDs) remains unclear. We report that a long noncoding RNA (lncRNA), H19, associates with dystrophin and inhibits E3-ligase-dependent polyubiquitination at Lys 3584 (referred to as Ub-DMD) and its subsequent protein degradation. In-frame deletions in BMD and a DMD non-silent mutation (C3340Y) resulted in defects in the ability of the protein to interact with H19, which caused elevated Ub-DMD levels and dystrophin degradation. Read More

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November 2020

Presentation and management of congenital myasthenic syndrome with a homozygous Agrin variant (Pro1448Leu).

Clin Neurol Neurosurg 2020 Dec 2;199:106277. Epub 2020 Oct 2.

Department of Neurology, University of Missouri, CE515, CS & E Building, Columbia, MO 65212, United States.

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December 2020

Circulating Biomarkers of Accelerated Sarcopenia in Respiratory Diseases.

Biology (Basel) 2020 Oct 3;9(10). Epub 2020 Oct 3.

Department of Cardiology, Al Qassimi Hospital, Sharjah 27272, UAE.

Skeletal muscle dysfunction is a critical finding in many respiratory diseases. However, a definitive biomarker to assess muscle decline in respiratory diseases is not known. We analyzed the association of plasma levels of glycoprotein Dickkopf-3 (Dkk-3), c-terminal agrin fragment-22 (CAF22) and microRNAs miR-21, miR-134a, miR-133 and miR-206 with hand-grip strength (HGS) and appendicular skeletal mass index (ASMI) in male, 54-73-year-old patients with chronic obstructive pulmonary diseases (COPD), asthma or pulmonary TB ( = 83-101/group). Read More

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October 2020

Doubling down on osteoarthritis.

Katie Kingwell

Nat Rev Drug Discov 2020 11;19(11):753

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November 2020

Myasthenia Gravis With Antibodies Against Muscle Specific Kinase: An Update on Clinical Features, Pathophysiology and Treatment.

Front Mol Neurosci 2020 2;13:159. Epub 2020 Sep 2.

Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom.

Muscle Specific Kinase myasthenia gravis (MuSK-MG) is an autoimmune disease that impairs neuromuscular transmission leading to generalized muscle weakness. Compared to the more common myasthenia gravis with antibodies against the acetylcholine receptor (AChR), MuSK-MG affects mainly the bulbar and respiratory muscles, with more frequent and severe myasthenic crises. Treatments are usually less effective with the need for prolonged, high doses of steroids and other immunosuppressants to control symptoms. Read More

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September 2020

POMK regulates dystroglycan function via LARGE1-mediated elongation of matriglycan.

Elife 2020 09 25;9. Epub 2020 Sep 25.

Howard Hughes Medical Institute, Senator Paul D. Wellstone Muscular Dystrophy Specialized Research Center, Department of Molecular Physiology and Biophysics and Department of Neurology, Roy J. and Lucille A. Carver College of Medicine, The University of Iowa, Iowa City, United States.

Matriglycan [-GlcA-β1,3-Xyl-α1,3-] serves as a scaffold in many tissues for extracellular matrix proteins containing laminin-G domains including laminin, agrin, and perlecan. Like-acetyl-glucosaminyltransferase 1 (LARGE1) synthesizes and extends matriglycan on α-dystroglycan (α-DG) during skeletal muscle differentiation and regeneration; however, the mechanisms which regulate matriglycan elongation are unknown. Here, we show that Protein -Mannose Kinase (POMK), which phosphorylates mannose of core M3 (GalNAc-β1,3-GlcNAc-β1,4-Man) preceding matriglycan synthesis, is required for LARGE1-mediated generation of full-length matriglycan on α-DG (~150 kDa). Read More

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September 2020

Corrigendum: Agrin-Mediated Cardiac Regeneration: Some Open Questions.

Front Bioeng Biotechnol 2020 21;8:935. Epub 2020 Aug 21.

School of Biochemistry, University of Bristol, Bristol, United Kingdom.

[This corrects the article on p. 594 in vol. 8, PMID: 32612983. Read More

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Acute Myasthenia Crisis: A Critical Emergency Department Differential.

Cureus 2020 Aug 15;12(8):e9760. Epub 2020 Aug 15.

Emergency Medicine, University of Central Florida College of Medicine/Hospital Corporation of America Healthcare Graduate Medical Education Consortium of Greater Orlando, Orlando, USA.

Myasthenia gravis (MG) is the most common autoimmune disorder of the neuromuscular junction (NMJ). It is caused by autoantibodies blocking acetylcholine receptors (AChRs) or structural receptors of the NMJ: agrin, LRP4, and MuSK. These antibodies can block, change, or destroy AChRs or structural proteins of the NMJ, preventing the binding of ACh and therefore, muscle contractions. Read More

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Agrin induces long-term osteochondral regeneration by supporting repair morphogenesis.

Sci Transl Med 2020 09;12(559)

Centre for Experimental Medicine and Rheumatology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London EC1M 6BQ, UK.

Cartilage loss leads to osteoarthritis, the most common cause of disability for which there is no cure. Cartilage regeneration, therefore, is a priority in medicine. We report that agrin is a potent chondrogenic factor and that a single intraarticular administration of agrin induced long-lasting regeneration of critical-size osteochondral defects in mice, with restoration of tissue architecture and bone-cartilage interface. Read More

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September 2020