GBP3
Description
The GBP3 (guanylate binding protein 3) is a protein-coding gene located on chromosome 1.
GBP3 is an interferon (IFN)-inducible GTPase that plays a crucial role in innate immunity against a wide array of bacterial, viral, and protozoan pathogens. It efficiently hydrolyzes GTP, primarily producing GDP rather than GMP. Upon infection, GBP3 localizes to pathogen-containing vacuoles or vacuole-escaped bacteria, acting as a positive regulator of inflammasome assembly by facilitating the release of inflammasome ligands from bacteria. This occurs through the lysis of pathogen-containing vacuoles, releasing pathogens into the cytosol. Subsequently, GBP3 promotes the recruitment of proteins involved in bacterial cytolysis, leading to the liberation of ligands detected by inflammasomes. These ligands include lipopolysaccharide (LPS), which activates the non-canonical CASP4/CASP11 inflammasome, and double-stranded DNA (dsDNA), which activates the AIM2 inflammasome. GBP3 has demonstrated antiviral activity against influenza virus, showing the most prominent antiviral activity in epithelial cells. GBP3 forms heterodimers with other members of the GBP family, including GBP1, GBP2, and GBP5, and dimerization regulates its subcellular localization.
GBP3 is also known as -.
Associated Diseases
- isolated agammaglobulinemia
- recurrent infections associated with rare immunoglobulin isotypes deficiency
- mannose-binding lectin deficiency
- hyper-IgM syndrome type 2
- MHC class II deficiency
- immunodeficiency due to ficolin3 deficiency
- severe combined immunodeficiency due to IKK2 deficiency
- common variable immunodeficiency
- autosomal recessive severe congenital neutropenia due to JAGN1 deficiency
- congenital sucrase-isomaltase deficiency
- cancer
- recurrent Neisseria infections due to factor D deficiency
- immunodeficiency, common variable, 7
- immunodeficiency 19
- cryptosporidiosis-chronic cholangitis-liver disease syndrome
- immunodeficiency, common variable, 4
