Abstract
Neuromyelitis Optica is an inflammatory neurodegenerative demyelinating autoimmune channelopathy affecting brain and spinal cord, for which Aquaporin-4(AQP4) water channels are the main target. Discovery of the AQP4 immunoglobulin (IGG) antibody in 2004 was a major step for understanding the disease which helped to distinguish from multiple sclerosis and other demyelinating diseases. Aquaporin -4 (AQP4) is a water channel protein expressed in two major isoforms which includes a short isoform M1 and a isoform M23 , AQP4 is expressed heavily on astrocyte endfeet end process at blood brain barrier, nodes of Ranvier and neuronal synapses processes in spinal cord, optic nerves , brain stem, thalamus ,hypothalamus, dorsal medulla at area postrema, diencephalon, subcortical white matter, gray and white matter in spinal cord, periaqueductal and periventricular region.
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