| Background | Pyrin (MEFV) is a ~86 kDa protein. Involved in the regulation of innate immunity and the inflammatory response in response to IFNG/IFN-gamma. 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. 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. 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. 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. 3. Yu, JW. et al. (2006) Cell death and differentiation 13, 236-49. PMID: 160378254. Chae, JJ. et al. (2006) Proceedings of the National Academy of Sciences of the United States of America 103, 9982-7. PMID: 167854465. Papin, S. et al. (2007) Cell death and differentiation 14, 1457-66. PMID: 174314228. Waite, AL. et al. (2009) Experimental biology and medicine (Maywood, N.J.) 234, 40-52. PMID: 1910955410. Kimura, T. et al. (2015) The Journal of cell biology 210, 973-89. PMID: 26347139 |
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