GBP7
Description
The GBP7 (guanylate binding protein 7) is a protein-coding gene located on chromosome 1.
GBP7 is an interferon (IFN)-inducible GTPase that plays a key role in the innate immune response against various bacterial, viral, and protozoan pathogens. It hydrolyzes GTP to GMP in two consecutive steps, primarily utilizing GTP as a substrate. Upon infection, GBP7 is recruited to pathogen-containing vacuoles or escaped bacteria, acting as a positive regulator of inflammasome assembly by facilitating the release of inflammasome ligands from these pathogens. This process involves promoting the lysis of pathogen-containing vacuoles, releasing the pathogens into the cytosol. Subsequently, GBP7 aids in recruiting proteins responsible for bacterial cytolysis, liberating ligands like lipopolysaccharide (LPS), which activates the non-canonical CASP4/CASP11 inflammasome, or double-stranded DNA (dsDNA), which activates the AIM2 inflammasome. Furthermore, GBP7 supports IFN-gamma-mediated host defense against bacterial infections by regulating oxidative responses and bacteriolytic peptide generation. It may also contribute to NADPH oxidase assembly on phagosomal membranes by acting as a bridge between NADPH oxidase cytosolic subunits (NCF2-NCF4) and membrane subunits (CYBA-CYBB). In collaboration with GBP1, GBP7 participates in trafficking monoubiquinated protein cargo to autolysosomes, generating ubiquitin-derived antimicrobial peptides. Interestingly, GBP7 facilitates influenza A virus replication by inhibiting the activation of NF-kappaB and JAK-STAT signaling pathways and suppressing the expression of type I, type III interferons, and pro-inflammatory cytokines. It provides protection against several pathogens, including bacterial pathogens like Listeria monocytogenes and Mycobacterium bovis BCG, as well as the protozoan pathogen Toxoplasma gondii. GBP7 is crucial for disrupting the parasitophorous vacuole formed during T.gondii infection, leading to the subsequent elimination of the parasite.
GBP7 is also known as GBP4L.
Associated Diseases
- recurrent Neisseria infections due to factor D deficiency
- immunodeficiency 28
- isolated asymptomatic elevation of creatine phosphokinase
- varicella, severe recurrent
- candidiasis, familial, 4
- plasma fibronectin deficiency
- type II complement component 8 deficiency
- mannose-binding lectin deficiency
- immunodeficiency due to ficolin3 deficiency
- pentosuria
- myopathy due to calsequestrin and SERCA1 protein overload
- autosomal recessive severe congenital neutropenia due to JAGN1 deficiency
- X-linked severe congenital neutropenia
- metabolic myopathy due to lactate transporter defect
- common variable immunodeficiency
