20 results match your criteria summary agrin

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Ocular myasthenia gravis: updates on an elusive target.

Curr Opin Neurol 2020 02;33(1):55-61

Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA.

Purpose Of Review: Ocular myasthenia gravis (OMG) is a complex condition with heterogenous phenotypes and ill-defined diagnostic criteria. Understanding concomitant risk factors and autoimmune serology can help inform prognosis for generalization and guide treatment.

Recent Findings: Although antibodies to acetylcholine receptors or muscle-specific kinase likely increase risk of generalization, they are less frequent in OMG. Read More

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February 2020

The clinical spectrum of the congenital myasthenic syndrome resulting from COL13A1 mutations.

Brain 2019 06;142(6):1547-1560

Department of Neuropediatrics and Muscle Disorders, Medical Center-University of Freiburg, Faculty of Medicine, Freiburg, Germany.

Next generation sequencing techniques were recently used to show mutations in COL13A1 cause synaptic basal lamina-associated congenital myasthenic syndrome type 19. Animal studies showed COL13A1, a synaptic extracellular-matrix protein, is involved in the formation and maintenance of the neuromuscular synapse that appears independent of the Agrin-LRP4-MuSK-DOK7 acetylcholine receptor clustering pathway. Here, we report the phenotypic spectrum of 16 patients from 11 kinships harbouring homozygous or heteroallelic mutations in COL13A1. Read More

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MYO9A deficiency in motor neurons is associated with reduced neuromuscular agrin secretion.

Hum Mol Genet 2018 04;27(8):1434-1446

John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne NE1 3BZ, UK.

Congenital myasthenic syndromes (CMS) are a group of rare, inherited disorders characterized by compromised function of the neuromuscular junction, manifesting with fatigable muscle weakness. Mutations in MYO9A were previously identified as causative for CMS but the precise pathomechanism remained to be characterized. On the basis of the role of MYO9A as an actin-based molecular motor and as a negative regulator of RhoA, we hypothesized that loss of MYO9A may affect the neuronal cytoskeleton, leading to impaired intracellular transport. Read More

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Natural product HTP screening for attenuation of cytokine-induced neutrophil chemo attractants (CINCs) and NO2- in LPS/IFNγ activated glioma cells.

J Neuroimmunol 2017 01 1;302:10-19. Epub 2016 Dec 1.

College of Pharmacy and Pharmaceutical Sciences, Florida A&M University, Tallahassee, FL 32307, United States. Electronic address:

Chronic and acute central nervous system (CNS) inflammation are contributors toward neurological injury associated with head trauma, stroke, infection, Parkinsons or Alzheimers disease. CNS inflammatory illnesses can also contribute toward risk of developing glioblastoma multiforme (GBM). With growing public interest in complementary and alternative medicines (CAMs), we conduct a high throughput (HTP) screening of >1400 natural herbs, plants and over the counter (OTC) products for anti-inflammatory effects on lipopolysaccharide (LPS)/interferon gamma (IFNγ) activated C6 glioma cells. Read More

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January 2017

Neuromuscular junction degeneration in muscle wasting.

Curr Opin Clin Nutr Metab Care 2016 May;19(3):177-81

aInterdisciplinary Center for Neuroscience, University of Heidelberg, Heidelberg bInstitute of Molecular and Cell Biology, Mannheim University of Applied Science, Mannheim cInstitute of Toxicology and Genetics, Karlsruhe Institute of Technology, Karlsruhe, Germany dDepartment of Kinesiology and Health Sciences, The College of William and Mary, Williamsburg, Virginia, USA eDepartment of Biomedical Science, University of Padua fVenetian Institute of Molecular Medicine (VIMM), Padua, Italy.

Purpose Of Review: Denervation is a hallmark of age-related and other types of muscle wasting. This review focuses on recent insights and current viewpoints regarding the mechanisms and clinical relevance of maintaining the neuromuscular junction to counteract muscle wasting resulting from aging or neural disease/damage.

Recent Findings: Activity-dependent regulation of autophagy, the agrin-muscle specific kinase-Lrp4 signaling axis, and sympathetic modulation are principal mechanisms involved in stabilizing the neuromuscular junction. Read More

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Proteoglycans in liver cancer.

World J Gastroenterol 2016 Jan;22(1):379-93

Kornélia Baghy, Péter Tátrai, Eszter Regős, Ilona Kovalszky, First Department of Pathology and Experimental Cancer Research, Semmelweis University, H1085 Budapest, Hungary.

Proteoglycans are a group of molecules that contain at least one glycosaminoglycan chain, such as a heparan, dermatan, chondroitin, or keratan sulfate, covalently attached to the protein core. These molecules are categorized based on their structure, localization, and function, and can be found in the extracellular matrix, on the cell surface, and in the cytoplasm. Cell-surface heparan sulfate proteoglycans, such as syndecans, are the primary type present in healthy liver tissue. Read More

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January 2016

Effects of a One-Year Physical Activity Program on Serum C-Terminal Agrin Fragment (CAF) Concentrations among Mobility-Limited Older Adults.

J Nutr Health Aging 2015 Nov;19(9):922-7

Thomas W. Buford, PhD, Translational Exercise, Aging, and Muscle Laboratory, Department of Aging and Geriatric Research, University of Florida, Gainesville, FL 32611, Telephone: 352-273-5918, Fax: 352-273-5920, Email:

Objectives: C-terminal Agrin Fragment (CAF) has been proposed as a potential circulating biomarker for predicting changes in physical function among older adults. To determine the effect of a one-year PA intervention on changes in CAF concentrations and to evaluate baseline and longitudinal associations between CAF concentrations and indices of physical function.

Design: Ancillary study to the Lifestyle Interventions and Independence for Elders Pilot (LIFE-P), a multi-site randomized clinical trial designed to evaluate the effects of chronic exercise on the physical function of older adults at risk for mobility disability. Read More

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November 2015

Factors controlling permeability of the blood-brain barrier.

Cell Mol Life Sci 2016 Jan 24;73(1):57-77. Epub 2015 Sep 24.

Department of Pharmacology and Toxicology, School of Pharmacy, University of Kansas, 1251 Wescoe Hall Drive, Malott Hall 5044, Lawrence, KS, 66045, USA.

As the primary protective barrier for neurons in the brain, the blood-brain barrier (BBB) exists between the blood microcirculation system and the brain parenchyma. The normal BBB integrity is essential in protecting the brain from systemic toxins and maintaining the necessary level of nutrients and ions for neuronal function. This integrity is mediated by structural BBB components, such as tight junction proteins, integrins, annexins, and agrin, of a multicellular system including endothelial cells, astrocytes, pericytes, etc. Read More

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January 2016

An update on laboratory diagnosis in myasthenia gravis.

Clin Chim Acta 2015 Sep 1;449:43-8. Epub 2015 Aug 1.

Division of Neurology, Department of Medicine, UBC Vancouver, Canada.

This review describes the state of the art for the use of laboratory testing in myasthenia gravis. The review brings a detailed description of the different clinical forms of auto-immune myasthenia and of the Lambert Eaton Myasthenic Syndrome (LEMS). They stress the differences between the different forms of acquired (auto-immune) myasthenia. Read More

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September 2015

An update on laboratory diagnosis in myasthenia gravis.

Clin Chim Acta 2015 Apr 15;444:126-31. Epub 2015 Feb 15.

Division of Neurology, Department of Medicine, UBC Vancouver, Canada.

This review describes the state of the art for the use of laboratory testing in myasthenia gravis. The review brings a detailed description of the different clinical forms of auto-immune myasthenia and of the Lambert Eaton Myasthenic Syndrome (LEMS). The stress the differences between the different forms of acquired (auto-immune) myasthenia. Read More

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Muscle-wide secretion of a miniaturized form of neural agrin rescues focal neuromuscular innervation in agrin mutant mice.

Proc Natl Acad Sci U S A 2008 Aug 6;105(32):11406-11. Epub 2008 Aug 6.

Biozentrum and Institute of Physiology, Department of Biomedicine, University of Basel, Klingelbergstrasse 70, 4056 Basel, Switzerland.

Agrin and its receptor MuSK are required for the formation of the postsynaptic apparatus at the neuromuscular junction (NMJ). In the current model the local deposition of agrin by the nerve and the resulting local activation of MuSK are responsible for creating and maintaining the postsynaptic apparatus including clusters of acetylcholine receptors (AChRs). Concomitantly, the release of acetylcholine (ACh) and the resulting depolarization disperses those postsynaptic structures that are not apposed by the nerve and thus not stabilized by agrin-MuSK signaling. Read More

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Revisiting the glomerular charge barrier in the molecular era.

Curr Opin Nephrol Hypertens 2008 Jul;17(4):393-8

Renal Division, Washington University School of Medicine, St. Louis, Missouri 63110, USA.

Purpose Of Review: The glomerular filtration barrier consists of fenestrated glomerular endothelium, podocyte foot processes/slit diaphragms, and intervening glomerular basement membrane. Its characterization as both a size and charge-selective barrier emerged from studies conducted decades ago. The charge selectivity phenomenon is receiving renewed attention now that the identities and mechanisms of synthesis of relevant molecules are known. Read More

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Synapse loss in cortex of agrin-deficient mice after genetic rescue of perinatal death.

J Neurosci 2007 Jul;27(27):7183-95

Biozentrum, University of Basel, CH-4056 Basel, Switzerland.

Agrin-deficient mice die at birth because of aberrant development of the neuromuscular junctions. Here, we examined the role of agrin at brain synapses. We show that agrin is associated with excitatory but not inhibitory synapses in the cerebral cortex. Read More

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Identification of developmentally regulated expression of MuSK in astrocytes of the rodent retina.

J Neurochem 2006 Oct 8;99(2):450-7. Epub 2006 Aug 8.

Institut für Biochemie, Universität Erlangen-Nürnberg, Erlangen, Germany.

One of the master regulators of postsynaptic neuromuscular synaptogenesis is the muscle-specific receptor tyrosine kinase (MuSK). In mammals prominent MuSK expression is believed to be restricted to skeletal muscle. Upon activation by nerve-derived agrin MuSK-dependent signalling participates in both the induction of genes encoding postsynaptic components and aggregation of nicotinic acetylcholine receptors (AChR) in the subsynaptic muscle membrane. Read More

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October 2006

Overexpression of mini-agrin in skeletal muscle increases muscle integrity and regenerative capacity in laminin-alpha2-deficient mice.

FASEB J 2005 Jun;19(8):934-42

Biozentrum, University of Basel, Basel, Switzerland.

Mutations in the gene encoding the alpha2 subunit of laminins cause the severe "merosin-deficient congenital muscular dystrophy" (MDC1A). We have recently shown that overexpression of a miniaturized form of the molecule agrin (mini-agrin) counteracts the disease in dy(W)/dy(W) mice, a model for MDC1A. However, these mice express some residual truncated laminin-alpha2, suggesting that the observed amelioration might be due to mini-agrin's presenting the residual laminin-alpha2 to its receptors. Read More

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Altered expression of NDST-1 messenger RNA in puromycin aminonucleoside nephrosis.

J Lab Clin Med 2004 Feb;143(2):106-14

Research Institute, International Medical Center of Japan, Tokyo, Japan.

Sulfated portions of glycosaminoglycan (GAG) side chains in heparan sulfate proteoglycan (HSPG) are thought to play an important role in charge-dependent selectivity of glomerular filtration against plasma proteins. Heparan sulfate N-acetylglucosamine N-deacetylase/adenosine 3'-phosphate 5'-phosphosulfate: unsubstituted glucosamine N-sulfotransferase (NDST) is the key enzyme regulating sulfation of GAG chains. In this study we investigated transcriptional expression of NDST-1, 1 of 4 isozymes of NDST, in glomeruli of rats with puromycin aminonucleoside (PAN) nephrosis. Read More

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February 2004

The heparan sulfate proteoglycan agrin modulates neurite outgrowth mediated by FGF-2.

J Neurobiol 2003 Jun;55(3):261-77

Department of Molecular Biomedical Sciences, College of Veterinary Medicine, North Carolina State University, 4700 Hillsborough Street, Raleigh, North Carolina 27606, USA.

Although the role of agrin in the formation of the neuromuscular junction is well established, other functions for agrin have remained elusive. The present study was undertaken to assess the role of agrin in neurite outgrowth mediated by the heparin-binding growth factor basic fibroblast growth factor (FGF-2), which we have shown previously to bind to agrin with high affinity and that has been shown to mediate neurite outgrowth from a number of neuronal cell types. Using both an established neuronal cell line, PC12 cells, and primary chick retina neuronal cultures, we find that agrin potentiates the ability of FGF-2 to stimulate neurite outgrowth. Read More

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Goto-Kakizaki rat is protected from proteinuria after induction of anti-Thy1 nephritis.

Am J Kidney Dis 2002 May;39(5):985-1000

Institute of Nephrology, University of Wales College of Medicine, Heath Park, Cardiff, Wales.

Hyperglycemia, although necessary, alone is insufficient for the development of progressive diabetic nephropathy. Two factors implicated in its pathogenesis are mesangial cell activation and/or proliferation and monocyte/macrophage influx. We have shown that prolonged hyperglycemia in the Goto-Kakizaki (GK) rat is associated with renal structural changes similar to those in patients with diabetes before the onset of progressive nephropathy. Read More

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A neuronal inhibitory domain in the N-terminal half of agrin.

J Neurobiol 2002 Feb;50(2):164-79

Department of Molecular & Cellular Pharmacology, University of Miami School of Medicine, 33101, USA.

Agrin is required for appropriate pre- and postsynaptic differentiation of neuromuscular junctions. While agrin's ability to orchestrate postsynaptic differentiation is well documented, more recent experiments have suggested that agrin is also a "stop signal" for the presynaptic neuron, and that agrin has actions on neurons in the CNS. To elucidate the neuronal activities of agrin and to define the receptor(s) responsible for these functions, we have examined adhesions of neurons and their neurite-outgrowth responses to purified agrin in vitro. Read More

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February 2002

Electron microscopic structure of agrin and mapping of its binding site in laminin-1.

EMBO J 1998 Jan;17(2):335-43

Department of Pharmacology, Biozentrum, University of Basel, Klingelbergstrasse 70, CH-4056 Basel, Switzerland.

Agrin is a large, multidomain heparan sulfate proteoglycan that is associated with basement membranes of several tissues. Particular splice variants of agrin are essential for the formation of synaptic structures at the neuromuscular junction. The binding of agrin to laminin appears to be required for its localization to synaptic basal lamina and other basement membranes. Read More

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January 1998
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