Anti-MEFV Antibody Picoband® Fluoro488 Conjugated

MEFV (Mediterranean fever) is a human gene that provides instructions for making a protein called pyrin (also known as marenostrin) . Pyrin is produced in certain white blood cells (neutrophils, eosinophils and monocytes) that play a role in inflammation and in fighting infection. Inside these white blood cells, pyrin is found with thecytoskeleton, the structural framework that helps to define the shape, size, and movement of a cell. Pyrin's protein structure also allows it to interact with other molecules involved in fighting infection and in the inflammatory response. Although pyrin's function is not fully understood, it likely assists in keeping the inflammation process under control. Research indicates that pyrin helps regulate inflammation by interacting with the cytoskeleton. And Pyrin may the migration of white blood cells to sites of inflammation and stop or slow the inflammatory response when it is no longer needed.

A synthetic peptide corresponding to a sequence at the N-terminus of human MEFV, different from the related mouse sequence by eight amino acids, and from the related rat sequence by eleven amino acids.

Expressed in peripheral blood leukocytes, particularly in mature granulocytes and to a lesser extent in monocytes but not in lymphocytes. Detected in spleen, lung and muscle, probably as a result of leukocyte infiltration in these tissues. Not expressed in thymus, prostate, testis, ovary, small intestine, colon, heart, brain, placenta, liver, kidney, pancreas. Expression detected in several myeloid leukemic, colon cancer, and prostate cancer cell lines. .

Actin Binding Proteins, Actin, etc., Cytoskeleton, Cytoskeleton/ECM, Immunology, Innate Immunity, Macrophage/Inflammation, Microfilaments, Signal Transduction

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 (PubMed:16785446, PubMed:17431422, PubMed:26347139) . However, it may also have a positive effect in the inflammatory pathway. In different experimental systems, it has been shown to activate IL1B production (PubMed:16037825) . It has also been shown to be required for PSTPIP1-induced PYCARD oligomerization and for formation of inflammasomes. Recruits PSTPIP1 to inflammasomes, and is required for PSTPIP1 oligomerization (PubMed:10807793, PubMed:11468188, PubMed:17964261, PubMed:18577712, PubMed:19109554, PubMed:19584923) . .

1. Dogan H, Akdemir F, Tasdemir S, Atis O, Diyarbakir E, Yildirim R, Emet M, Ikbal M (2014) . A novel insertion mutation identified in exon 10 of the MEFV gene associated with Familial Mediterranean Fever. BMC Medical Genetics 15 (1) : 74. 2. Mansfield E, Chae JJ, Komarow HD, Brotz TM, Frucht DM, Aksentijevich I, Kastner DL (Aug 2001) . The familial Mediterranean fever protein, pyrin, associates with microtubules and colocalizes with actin filaments. Blood 98 (3) : 851–9.

Isoform 1: Cytoplasm, cytoskeleton. Cell projection, ruffle. Cell projection, lamellipodium. Nucleus. Cytoplasm . Cytoplasmic vesicle, autophagosome . Associated with microtubules and with the filamentous actin of perinuclear filaments and peripheral lamellar ruffles. In pre-apoptotic cells, colocalizes with PYCARD/ASC in large specks (inflammasomes) . In migrating monocytes, strongly polarized at the leading edge of the cell where it colocalizes with polymerizing actin and PYCARD/ASC.