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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.
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 ]
Astrogliosis (also known as astrocytosis or referred to as reactive astrogliosis) is an abnormal increase in the number of astrocytes due to the destruction of nearby neurons from central nervous system (CNS) trauma, infection, ischemia, stroke, autoimmune responses or neurodegenerative disease.
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]
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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.