IFNGR1 Rabbit Polyclonal Antibody (ER63732)

UNIPROT #

P15260

PROTEIN NAME

IFNGR1

SYNONYMS

Antiviral Protein Type II antibody;Antiviral protein, type 2 antibody;AVP type II antibody;AVP, type 2 antibody;CD 119 antibody;CD119 antibody;CD119 antigen antibody;CDw119 antibody;FLJ45734 antibody;IFN gamma R alpha antibody;IFN gamma R antibody;IFN gamma R1 antibody;IFN-gamma receptor 1 antibody;IFN-gamma-R1 antibody;IFNG R1 antibody;IFNGR 1 antibody;IFNGR antibody;IFNGR1 antibody;Immune interferon receptor 1 antibody;Immune interferon receptor for antibody;INGR1_HUMAN antibody;Interferon gamma receptor 1 antibody;Interferon gamma receptor alpha chain antibody;Interferon gamma receptor alpha chain precursor antibody

SUBCELLULAR LOCATION

Cell membrane.

FUNCTION

Receptor subunit for interferon gamma/INFG that plays crucial roles in antimicrobial, antiviral, and antitumor responses by activating effector immune cells and enhancing antigen presentation. Associates with transmembrane accessory factor IFNGR2 to form a functional receptor. Upon ligand binding, the intracellular domain of IFNGR1 opens out to allow association of downstream signaling components JAK1 and JAK2. In turn, activated JAK1 phosphorylates IFNGR1 to form a docking site for STAT1. Subsequent phosphorylation of STAT1 leads to dimerization, translocation to the nucleus, and stimulation of target gene transcription. STAT3 can also be activated in a similar manner although activation seems weaker. IFNGR1 intracellular domain phosphorylation also provides a docking site for SOCS1 that regulates the JAK-STAT pathway by competing with STAT1 binding to IFNGR1. A form of Mendelian susceptibility to mycobacterial disease, a rare condition caused by impairment of interferon-gamma mediated immunity. It is characterized by predisposition to illness caused by moderately virulent mycobacterial species, such as Bacillus Calmette-Guerin (BCG) vaccine, environmental non-tuberculous mycobacteria, and by the more virulent Mycobacterium tuberculosis. Other microorganisms rarely cause severe clinical disease in individuals with susceptibility to mycobacterial infections, with the exception of Salmonella which infects less than 50% of these individuals. Clinical outcome severity depends on the degree of impairment of interferon-gamma mediated immunity. Some patients die of overwhelming mycobacterial disease with lepromatous-like lesions in early childhood, whereas others develop, later in life, disseminated but curable infections with tuberculoid granulomas.