Linked Life Data

19699271

Source:http://linkedlifedata.com/resource/pubmed/id/19699271

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
2010-6-14
pubmed:abstractText
Neuromyelitis optica (NMO) is the first inflammatory autoimmune demyelinating disease of the CNS for which a specific tissue target molecule has been identified--the astrocytic water channel aquaporin-4 (AQP4). Immunological insights have propelled significant advances in understanding the clinical, radiologic and immunopathologic characteristics of the disease in the last 5 years. In this review, we describe features distinguishing CNS AQP4 autoimmunity from classical multiple sclerosis (MS). In NMO, disease attacks preferentially involve the optic nerves and spinal cord (hence the name), but neurological signs in the initial attack of AQP4 autoimmunity in children commonly involve the brain. A clinically validated serum biomarker, NMO-IgG, distinguishes relapsing CNS inflammatory demyelinating disorders related to NMO from MS. The NMO-IgG autoantibody is AQP4-specific. Clinical, radiological and immunopathological data support its role in the pathogenesis of NMO spectrum disorders. Lesions characteristic of NMO are distinct from MS: AQP4 and its coupled glutamate transporter, excitatory amino acid transporter 2 (EAAT2), are lost, with and without associated myelin loss, IgG, IgM and complement are deposited in a vasculocentric pattern, edema and inflammation are prominent. In vitro studies demonstrate that binding of NMO-IgG to astrocytic AQP4 initiates multiple potentially neuropathogenic mechanisms: complement activation, AQP4 and EAAT2 downregulation with disruption of water and glutamate homeostasis, enhanced blood-brain barrier permeability, plasma protein and granulocyte influx, and antibody-dependent cell-mediated cytotoxicity. Development of effective, and potentially curative, therapies requires validated models of the disease, in animals and cell culture systems.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
1873-7544
pubmed:author
pubmed:copyrightInfo
Copyright (c) 2010 IBRO. Published by Elsevier Ltd. All rights reserved.
pubmed:issnType
Electronic
pubmed:day
28
pubmed:volume
168
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1009-18
pubmed:meshHeading
pubmed:year
2010
pubmed:articleTitle
Neurological autoimmunity targeting aquaporin-4.
pubmed:affiliation
Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
pubmed:publicationType
Journal Article, Review, Research Support, Non-U.S. Gov't