The KCNJ10 antibody targets the KCNJ10 protein, encoded by the KCNJ10 gene, which belongs to the inwardly rectifying potassium (Kir) channel family. Kir4.1. the protein product, plays a critical role in maintaining potassium ion homeostasis and regulating membrane potential, particularly in the brain, kidneys, and inner ear. In the central nervous system, Kir4.1 is predominantly expressed in astrocytes, where it supports neuronal excitability, potassium buffering, and extracellular space volume regulation. In the kidneys, it facilitates potassium recycling in distal nephrons, aiding electrolyte balance.
KCNJ10 dysfunction is linked to disorders like EAST/SeSAME syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) and neurodegenerative conditions. Autoantibodies against Kir4.1 have been implicated in autoimmune neuroinflammatory diseases, including multiple sclerosis and neuromyelitis optica spectrum disorders, suggesting its role as a potential biomarker.
The KCNJ10 antibody is widely used in research to study Kir4.1 expression patterns, localization, and pathological mechanisms via techniques like immunohistochemistry, Western blotting, and immunofluorescence. It also aids in diagnosing autoimmune conditions by detecting anti-Kir4.1 antibodies in patient sera. Understanding KCNJ10's biology and its antibodies provides insights into potassium channel-related diseases and therapeutic targeting.