Sign in or Register   Sign in or Register
  |  

Mouse Anti-MEFV (AA 1-110) Recombinant Antibody (CBFYM-2038) (CBMAB-M2218-FY)

This product is mouse antibody that recognizes MEFV. The antibody CBFYM-2038 can be used for immunoassay techniques such as: ELISA, IF, WB.
See all MEFV antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYM-2038
Antibody Isotype
IgG2a, k
Application
ELISA, IF, WB

Basic Information

Immunogen
Recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: MAKTPSDHLL STLEELVPYD FEKFKFKLQN TSVQKEHSRI PRSQIQRARP VKMATLLVTY YGEEYAVQLT LQVLRAINQR LLAEELHRAA IQEYSTQENG TDDSAASSSL
Specificity
Human
Antibody Isotype
IgG2a, k
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 1-110

Target

Full Name
Mediterranean fever
Introduction
This gene encodes a protein, also known as pyrin or marenostrin, that is an important modulator of innate immunity. Mutations in this gene are associated with Mediterranean fever, a hereditary periodic fever syndrome.
Entrez Gene ID
4210
UniProt ID
O15553
Alternative Names
MEFV, Pyrin Innate Immunity Regulator; Marenostrin; Mediterranean Fever; TRIM20; MEF; Pyrin; FMF
Function
Involved in the regulation of innate immunity and the inflammatory response in response to IFNG/IFN-gamma (PubMed:10807793, PubMed:11468188, PubMed:17964261, PubMed:18577712, PubMed:19109554, PubMed:19584923, PubMed:16037825, PubMed:27030597, PubMed:28835462, PubMed:16785446, PubMed:17431422, PubMed:26347139).

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 (PubMed:16785446, PubMed:17431422, PubMed:26347139).

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 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 (PubMed:16037825, PubMed:27030597, PubMed:28835462).

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 (By similarity).

It is required for PSTPIP1-induced PYCARD/ASC oligomerization and inflammasome formation (PubMed:10807793, PubMed:11468188, PubMed:17964261, PubMed:18577712, PubMed:19109554, PubMed:19584923).

Recruits PSTPIP1 to inflammasomes, and is required for PSTPIP1 oligomerization (PubMed:10807793, PubMed:11468188, PubMed:17964261, PubMed:18577712, PubMed:19109554, PubMed:19584923).
Biological Process
Inflammatory response Source: UniProtKB
Innate immune response Source: GO_Central
Negative regulation of cytokine production involved in inflammatory response Source: UniProtKB
Negative regulation of inflammatory response Source: BHF-UCL
Negative regulation of interleukin-12 production Source: BHF-UCL
Negative regulation of interleukin-1 beta production Source: BHF-UCL
Negative regulation of macrophage inflammatory protein 1 alpha production Source: BHF-UCL
Negative regulation of NLRP3 inflammasome complex assembly Source: UniProtKB
Pattern recognition receptor signaling pathway Source: ComplexPortal
Positive regulation of autophagy Source: UniProtKB
Positive regulation of cysteine-type endopeptidase activity Source: UniProtKB
Positive regulation of interleukin-1 beta production Source: ComplexPortal
Protein ubiquitination Source: GO_Central
Pyroptosis Source: ComplexPortal
Pyroptosome complex assembly Source: UniProtKB
Regulation of gene expression Source: GO_Central
Regulation of interleukin-1 beta production Source: UniProtKB
Response to interferon-gamma Source: UniProtKB
Cellular Location
Isoform 1:
Nucleus
Cytoskeleton
Cytoplasm
Other locations
ruffle
lamellipodium
autophagosome
Note: Associated with microtubules and with the filamentous actin of perinuclear filaments and peripheral lamellar ruffles (PubMed:11468188). In pre-apoptotic cells, colocalizes with PYCARD/ASC in large specks (inflammasomes) (PubMed:11468188). In migrating monocytes, strongly polarized at the leading edge of the cell where it colocalizes with polymerizing actin and PYCARD/ASC (PubMed:11468188).
Isoform 2:
Nucleus
Involvement in disease
Familial Mediterranean fever, autosomal recessive (ARFMF):
A hereditary periodic fever syndrome characterized by recurrent episodic fever, serosal inflammation and pain in the abdomen, chest or joints. It is frequently complicated by reactive amyloidosis, which leads to renal failure and can be prophylactically treated with colchicine.
Familial Mediterranean fever, autosomal dominant (ADFMF):
A hereditary periodic fever syndrome characterized by periodic fever, serosal inflammation and pain in the abdomen, chest or joints as seen also in the autosomal recessive form of the disease. It is associated with reactive renal amyloidosis and characterized by colchicine unresponsiveness.
Pyrin-associated autoinflammatory disease (PAAND):
An autosomal dominant autoinflammatory disorder characterized by childhood onset of recurrent episodes of fever, neutrophilic dermatosis, myalgia and arthralgia. The neutrophilic dermatosis comprises a spectrum of clinical manifestations, including severe acne, sterile skin abscesses, pyoderma gangrenosum, and neutrophilic small-vessel vasculitis. Pathological examination of affected skin shows a dense, predominantly neutrophilic, vascular, perivascular, and interstitial infiltrate. PAAND has incomplete penetrance and variable expressivity.
PTM
Cleaved by CASP1 (Probable). The N-terminal cleavage product localizes to the nucleus as a filamentous network and to the cytoplasm, interacts more strongly with RELA and NFKBIA than the full-length protein, enhances the nuclear localization of RELA and induces NFKBIA proteolysis. The C-terminal cleavage product localizes to the cytoplasm (Probable).
Phosphorylation at Ser-242 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).
Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-MEFV (AA 1-110) Recombinant Antibody (CBFYM-2038)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare