Initial studies revealed a pathogenic effect of the humoral immune response against MOG (human MOG-Ab) these antibodies were able to induce the death of MOG-expressing cells as well as natural killer cell-mediated cell death, with the extent of cell damage dependent on antibody levels. Furthermore, purified IgG from MOG-IgG–positive patients, when incubated with oligodendrocytes in vitro, led to obvious cytoskeletal disorganization, further suggesting functional pathogenicity. In humans, high-titer MOG-immunoglobulin G (MOG-IgG) levels in serum samples seem to efficiently activate the complement cascade in vitro. Key words: Myelin oligodendrocyte glycoprotein, Demyelinating diseases, Neuromyelitis optica, Optic neuritis, Acute disseminated encephalomyelitis This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases.
0 kommentar(er)
