anti-Fibrillin 1 antibody
anti-Fibrillin 1 antibody for IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Mouse
|产品描述||Rabbit Polyclonal antibody recognizes Fibrillin 1|
|抗原||Synthetic peptide between aa. 2780-2860 of Human Fibrillin 1.|
|別名||ECTOL1; MFS1; WMS; SGS; SSKS; MASS; GPHYSD2; WMS2; ACMICD; OCTD; Fibrillin-1; FBN|
|应用说明||IHC-P: Antigen Retrieval: Incubated in Tris-EDTA buffer (pH 8.0) with high-pressure and temperature.
* The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist.
|纯化||Affinity purification with immunogen.|
|缓冲液||PBS, 0.02% Sodium azide, 50% Glycerol and 0.5% BSA.|
|抗菌剂||0.02% Sodium azide|
|稳定剂||50% Glycerol and 0.5% BSA|
|存放说明||For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use.|
|注意事项||For laboratory research only, not for drug, diagnostic or other use.|
|背景介绍||This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. [provided by RefSeq, Jul 2008]|
|生物功能||Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. [UniProt]|
|细胞定位||Secreted. Note=Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin (PubMed:24982166). Asprosin: Secreted. Note=Secreted into the plasma. Fibrillin-1: Secreted, extracellular space, extracellular matrix. [UniProt]|
|翻译后修饰||Fibrillin-1: Cleavage of N- and C-terminus by furin is required for incorporation into the extracellular matrix and assembly into microfibrils (PubMed:27026396). The C-terminus, which corresponds to the Asprosin chain, was initially thought to constitute a propeptide (PubMed:24982166). Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin, an essential step for incorporation of Fibrillin-1 into the nascent microfibrils (PubMed:24982166).
Fibrillin-1: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils. [UniProt]
检测图片 (2) Click the Picture to Zoom In
ARG66654 anti-Fibrillin 1 antibody WB image
Western blot: Mouse liver lysate stained with ARG66654 anti-Fibrillin 1 antibody.
ARG66654 anti-Fibrillin 1 antibody IHC-P image
Immunohistochemistry: Paraffin-embedded Human breast carcinoma tissue stained with ARG66654 anti-Fibrillin 1 antibody. The picture on the right is blocked with the synthetic peptide.