<?xml version="1.0" encoding="UTF-8"?><xml><records><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Greenberg, S. A.</style></author><author><style face="normal" font="default" size="100%">Pinkus, J. L.</style></author><author><style face="normal" font="default" size="100%">Pinkus, G. S.</style></author><author><style face="normal" font="default" size="100%">Burleson, T.</style></author><author><style face="normal" font="default" size="100%">Sanoudou, D.</style></author><author><style face="normal" font="default" size="100%">Tawil, R.</style></author><author><style face="normal" font="default" size="100%">Barohn, R. J.</style></author><author><style face="normal" font="default" size="100%">Saperstein, D. S.</style></author><author><style face="normal" font="default" size="100%">Briemberg, H. R.</style></author><author><style face="normal" font="default" size="100%">Ericsson, M.</style></author><author><style face="normal" font="default" size="100%">Park, P.</style></author><author><style face="normal" font="default" size="100%">Amato, A. A.</style></author></authors></contributors><titles><title><style face="normal" font="default" size="100%">Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis</style></title><secondary-title><style face="normal" font="default" size="100%">Ann NeurolAnn NeurolAnn Neurol</style></secondary-title><alt-title><style face="normal" font="default" size="100%">Annals of neurology</style></alt-title><short-title><style face="normal" font="default" size="100%">Annals of neurologyAnnals of neurology</style></short-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">Adult</style></keyword><keyword><style  face="normal" font="default" size="100%">Aged</style></keyword><keyword><style  face="normal" font="default" size="100%">Blood Vessels/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">CD3 Complex/biosynthesis</style></keyword><keyword><style  face="normal" font="default" size="100%">CD4 Antigens/biosynthesis</style></keyword><keyword><style  face="normal" font="default" size="100%">Dendritic Cells/pathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Dermatomyositis/*immunology/pathology/*physiopathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Female</style></keyword><keyword><style  face="normal" font="default" size="100%">Gene Expression Regulation/physiology</style></keyword><keyword><style  face="normal" font="default" size="100%">GTP-Binding Proteins/biosynthesis</style></keyword><keyword><style  face="normal" font="default" size="100%">Humans</style></keyword><keyword><style  face="normal" font="default" size="100%">Immunohistochemistry</style></keyword><keyword><style  face="normal" font="default" size="100%">Interferon-alpha/*physiology</style></keyword><keyword><style  face="normal" font="default" size="100%">Interferon-beta/*physiology</style></keyword><keyword><style  face="normal" font="default" size="100%">Lectins, C-Type/biosynthesis</style></keyword><keyword><style  face="normal" font="default" size="100%">Male</style></keyword><keyword><style  face="normal" font="default" size="100%">Membrane Glycoproteins</style></keyword><keyword><style  face="normal" font="default" size="100%">Microscopy, Immunoelectron</style></keyword><keyword><style  face="normal" font="default" size="100%">Middle Aged</style></keyword><keyword><style  face="normal" font="default" size="100%">Muscle Fibers, Skeletal/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Muscle, Skeletal/pathology/physiopathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Myositis, Inclusion Body/pathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Myxovirus Resistance Proteins</style></keyword><keyword><style  face="normal" font="default" size="100%">Nerve Tissue Proteins/biosynthesis</style></keyword><keyword><style  face="normal" font="default" size="100%">Neuromuscular Diseases/pathology/physiopathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Oligonucleotide Array Sequence Analysis</style></keyword><keyword><style  face="normal" font="default" size="100%">Promoter Regions, Genetic/genetics</style></keyword><keyword><style  face="normal" font="default" size="100%">Prospective Studies</style></keyword><keyword><style  face="normal" font="default" size="100%">Receptors, Immunologic</style></keyword><keyword><style  face="normal" font="default" size="100%">Reverse Transcriptase Polymerase Chain Reaction</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2005</style></year><pub-dates><date><style  face="normal" font="default" size="100%">May</style></date></pub-dates></dates><number><style face="normal" font="default" size="100%">5</style></number><volume><style face="normal" font="default" size="100%">57</style></volume><pages><style face="normal" font="default" size="100%">664-78</style></pages><isbn><style face="normal" font="default" size="100%">0364-5134 (Print)0364-5134 (Linking)</style></isbn><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">Dermatomyositis has been modeled as an autoimmune disease largely mediated by the adaptive immune system, including a local humorally mediated response with B and T helper cell muscle infiltration, antibody and complement-mediated injury of capillaries, and perifascicular atrophy of muscle fibers caused by ischemia. To further understand the pathophysiology of dermatomyositis, we used microarrays, computational methods, immunohistochemistry and electron microscopy to study muscle specimens from 67 patients, 54 with inflammatory myopathies, 14 with dermatomyositis. In dermatomyositis, genes induced by interferon-alpha/beta were highly overexpressed, and immunohistochemistry for the interferon-alpha/beta inducible protein MxA showed dense staining of perifascicular, and, sometimes all myofibers in 8/14 patients and on capillaries in 13/14 patients. Of 36 patients with other inflammatory myopathies, 1 patient had faint MxA staining of myofibers and 3 of capillaries. Plasmacytoid dendritic cells, potent CD4+ cellular sources of interferon-alpha, are present in substantial numbers in dermatomyositis and may account for most of the cells previously identified as T helper cells. In addition to an adaptive immune response, an innate immune response characterized by plasmacytoid dendritic cell infiltration and interferon-alpha/beta inducible gene and protein expression may be an important part of the pathogenesis of dermatomyositis, as it appears to be in systemic lupus erythematosus.</style></abstract><accession-num><style face="normal" font="default" size="100%">15852401</style></accession-num><notes><style face="normal" font="default" size="100%">Greenberg, Steven APinkus, Jack LPinkus, Geraldine SBurleson, TravisSanoudou, DespinaTawil, RabiBarohn, Richard JSaperstein, David SBriemberg, Hannah REricsson, MariaPark, PeterAmato, Anthony AengR01NS43471/NS/NINDS NIH HHS/Research Support, N.I.H., ExtramuralResearch Support, U.S. Gov't, P.H.S.2005/04/27 09:00Ann Neurol. 2005 May;57(5):664-78. doi: 10.1002/ana.20464.</style></notes><auth-address><style face="normal" font="default" size="100%">Department of Neurology, Division of Neuromuscular Disease, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA. sagreenberg@partners.org</style></auth-address></record></records></xml>