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Autoimmune GFAP Astrocytopathy is an autoimmune disease in which the immune system of the patient attacks a protein of the nervous system called glial fibrillary acidic protein (GFAP). It was described in 2016 by researchers of the Mayo Clinic in the United States. [1]
Glial fibrillary acidic protein (GFAP) is a protein that is encoded by the GFAP gene in humans. [5] It is a type III intermediate filament (IF) protein that is expressed by numerous cell types of the central nervous system (CNS), including astrocytes [ 6 ] and ependymal cells during development. [ 7 ]
An autoantibody—glial fibrillary acidic protein (GFAP)—was found in 2016, in transverse myelitis and atypical NMO, leading to the concept of autoimmune GFAP astrocytopathy. [37] Other autoantibody being researched is flotillin. It has been found in seronegative NMO and some MS patients. [84]
Reactive astrogliosis is a spectrum of changes in astrocytes that occur in response to all forms of CNS injury and disease. Changes due to reactive astrogliosis vary with the severity of the CNS insult along a graduated continuum of progressive alterations in molecular expression, progressive cellular hypertrophy, proliferation and scar formation.
As MS is an active field for research, the list of auto-antibodies is not closed nor definitive. For example, some diseases like Autoimmune GFAP Astrocytopathy or variants of CIDP that affects the CNS (CIDP is the chronic counterpart of Guillain–Barré syndrome) could be included.
In 2016, it was identified in Mayo clinic an autoimmune form of myelitis due to the presence of anti-GFAP autoantibodies. Immunoglobulins directed against the α-isoform of glial fibrillary acidic protein (GFAP-IgG) predicted a special meningoencephalomyelitis termed autoimmune GFAP Astrocytopathy [ 10 ] that later was found also to be able to ...
Autoimmune GFAP astrocytopathy; Autoimmune hemolytic anemia; Autoimmune hypophysitis; Autoimmune inner ear disease; Autoimmune oophoritis; Autoimmune pancreatitis; Autoimmune polyendocrine syndrome; Autoimmune polyendocrine syndrome type 1; Autoimmune polyendocrine syndrome type 2; Autoimmune polyendocrine syndrome type 3; Autoimmune retinopathy
Type 1: Antigenically Ran2 +, GFAP +, FGFR3 +, A2B5 −, thus resembling the "type 1 astrocyte" of the postnatal day 7 rat optic nerve. These can arise from the tripotential glial restricted precursor cells (GRP), but not from the bipotential O2A/OPC (oligodendrocyte, type 2 astrocyte precursor, also called Oligodendrocyte progenitor cell ) cells.