The B box-type zinc finger interacts, possibly intramolecularly, with the pyrin domain; this may be an autoinhibitory mechanism released by PSTPIP1 binding.
Involved in the regulation of innate immunity and the inflammatory response in response to IFNG/IFN-gamma (PubMed:34471287). Organizes autophagic machinery by serving as a platform for the assembly of ULK1, Beclin 1/BECN1, ATG16L1, and ATG8 family members and recognizes specific autophagy targets, thus coordinating target recognition with assembly of the autophagic apparatus and initiation of autophagy (By similarity). Acts as an autophagy receptor for the degradation of several inflammasome components, including CASP1, NLRP1 and NLRP3, hence preventing excessive IL1B- and IL18-mediated inflammation (By similarity). However, it can also have a positive effect in the inflammatory pathway, acting as an innate immune sensor that triggers PYCARD/ASC specks formation, caspase-1 activation, and IL1B and IL18 production (By similarity). Together with AIM2, also acts as a mediator of pyroptosis, necroptosis and apoptosis (PANoptosis), an integral part of host defense against pathogens, in response to bacterial infection (PubMed:34471287). It is required for PSTPIP1-induced PYCARD/ASC oligomerization and inflammasome formation (By similarity). Recruits PSTPIP1 to inflammasomes, and is required for PSTPIP1 oligomerization (By similarity).
Phosphorylation at Ser-241 is required for the interaction with 14-3-3 proteins and down-regulation of pyrin pro-inflammatory activity.
Degraded along with the delivery of its substrates to autolysosomal compartments (at protein level).
Expressed in spleen peripheral blood granulocytes. Not expressed in lymphocytes, thymus, testis, ovary, heart, brain, lung, liver, kidney and muscle.
Pyrin, Mefv
Proteins
Immunology & Infectious Disease
86409Da
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