Interferon gamma receptor 1 (IFNGR1) (IFNGR1)

Interferon gamma receptor 1 (IFNGR1), also known as the alpha chain or CD119, is a transmembrane protein that serves as the primary binding component of the heterodimeric interferon-gamma (IFNγ) receptor complex [UniProt: P15260]. Upon binding its ligand, IFNGR1 associates with the accessory chain IFNGR2, triggering the activation of the Janus kinase (JAK1 and JAK2) and signal transducer and activator of transcription 1 (STAT1) pathway [NCBI Gene: 3459]. This signaling cascade is fundamental for macrophage activation, upregulating MHC class I and II molecules, and orchestrating the host's immune response against intracellular pathogens and tumors. Genetic deficiencies in IFNGR1 lead to Mendelian Susceptibility to Mycobacterial Diseases (MSMD), a condition characterized by severe infections from weakly virulent mycobacteria [StatPearls: MSMD]. Therapeutically, recombinant IFNγ (Interferon gamma-1b) is utilized as an agonist to enhance immune function in patients with chronic granulomatous disease, while the IFNγ/IFNGR1 axis is a target for inhibition in conditions like hemophagocytic lymphohistiocytosis (HLH) to curb excessive inflammation [DrugBank: DB00033].

Other names

CD119IFN-gamma receptor alpha chainIFN-gamma-R1Interferon gamma receptor subunit 1

Agonism of the receptor complex to stimulate the JAK-STAT signaling pathway for immunomodulation.