Briquilimab

Briquilimab, also known as JSP-191 or AMG-191, is a humanized IgG1 monoclonal antibody that targets CD117 (or c-Kit). Briquilimab blocks the interaction between CD117 receptor and stem cell factor. c-KIT is a proto-oncogene and a type III transmembrane receptor for mast cell growth factor, also known as stem cell factor. It plays an essential role in the regulation of cell survival and proliferation, as well as hematopoiesis, stem cell maintenance, gametogenesis, melanogenesis, and mast cell development, migration and function. Activating mutations in cKIT are associated with gastrointestinal stromal tumors, testicular seminoma, mast cell disease, melanoma, and acute myeloid leukemia, while inactivating mutations are associated with the genetic defect piebaldism. Briquilimab, also known as JSP-191 or AMG-191, is a humanized IgG1 monoclonal antibody that binds CD117. Briquilimab blocks the interaction between CD117 receptor and stem cell factor on various CD117 expressing tissues and has been under consideration in the context of diseases like evere combined immunodeficiency (SCID), myelodyplastic syndromes (MDS), acute myeloid leukemia (AML), chronic spontaneous urticarial (CSU), chronic inducible urticarial (CIndU) and asthema.