NLRP3 Recombinant Rabbit Monoclonal Antibody [SC06-23] (ET1610-93)

UNIPROT #

Q96P20

PROTEIN NAME

NLRP3

SYNONYMS

AGTAVPRL antibody;AII/AVP antibody;Angiotensin/vasopressin receptor AII/AVP like antibody;Angiotensin/vasopressin receptor AII/AVP-like antibody;C1orf7 antibody;Caterpiller protein 1.1 antibody;CIAS 1 antibody;CIAS1 antibody;CLR1.1 antibody;Cold autoinflammatory syndrome 1 antibody;Cold autoinflammatory syndrome 1 protein antibody;Cryopyrin antibody;Familial cold autoinflammatory syndrome antibody;FCAS antibody;FCU antibody;LRR and PYD domains-containing protein 3 antibody;Muckle-Wells syndrome antibody;MWS antibody;NACHT antibody;NACHT LRR and PYD containing protein 3 antibody;NALP 3 antibody;NALP3 antibody;NALP3_HUMAN antibody;NLRP3 antibody;PYPAF 1 antibody;PYPAF1 antibody;PYRIN containing APAF1 like protein 1 antibody;PYRIN-containing APAF1-like protein 1 antibody

SEQUENCE SIMILARITIES

Belongs to the NLRP family.

TISSUE SPECIFICITY

Predominantly expressed in macrophages. Also expressed in dendritic cells, B- and T-cells (at protein level). Expressed in LPS-treated granulocytes, but not in resting cells (at protein level). Expression in monocytes is very weak (at protein level). Expressed in stratified non-keratinizing squamous epithelium, including oral, esophageal and ectocervical mucosa and in the Hassall's corpuscles in the thymus. Also, detected in the stratified epithelium covering the bladder and ureter (transitional mucosa) (at protein level). Expressed in lung epithelial cells (at protein level). Expressed in chondrocytes. Expressed at low levels in resting osteoblasts.

POST-TRANSLATIONAL MODIFICATION

The disulfide bond in the pyrin domain might play a role in reactive oxygen species-mediated activation.; Ubiquitinated; undergoes both 'Lys-48'- and 'Lys-63'-linked polyubiquitination. Ubiquitination does not lead to degradation, but inhibits inflammasome activation (By similarity). Deubiquitination is catalyzed by BRCC3 and associated with NLRP3 activation and inflammasome assembly. This process can be induced by the activation of Toll-like receptors (by LPS), through a non-transcriptional pathway dependent on the mitochondrial production of reactive oxygen species, and by ATP.

SUBCELLULAR LOCATION

Cytoplasm, Inflammasome, Secreted, Nucleus, Endoplasmic reticulum.

FUNCTION

NLRP3 is expressed predominantly in macrophages and as a component of the inflammasome, detects products of damaged cells such as extracellular ATP and crystalline uric acid. Activated NLRP3 in turn triggers an immune response. Mutations in the NLRP3 gene are associated with a number of organ specific autoimmune diseases. NLRP3 is a component of the innate immune system that functions as a pattern recognition receptor (PRR) that recognizes pathogen-associated molecular patterns (PAMPs). NLRP3 belongs to the NOD-like receptor (NLR) subfamily of PRRs and NLRP3 together with the adaptor ASC protein PYCARD forms a caspase-1 activating complex known as the NLRP3 inflammasome. NLRP3 in the absence of activating signal is kept in an inactive state complexed with HSP90 and SGT1 in the cytoplasm. NLRP3 inflammasome detects danger signals such as crystalline uric acid and extracellular ATP released by damaged cells. These signals release HSP90 and SGT1 from and recruit ASC protein and caspase-1 to the inflammasome complex. Caspase-1 within the activated NLRP3 inflammasome complex in turn activates the inflammatory cytokine, IL-1β. The NLRP3 inflammasome appears to be activated by changes in intracellular potassium caused by potassium efflux from mechanosensitive ion channels located in the cell membrane