anti-Fibrillin 1 antibody
anti-Fibrillin 1 antibody for IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Mouse
|Product Description||Rabbit Polyclonal antibody recognizes Fibrillin 1|
|Tested Reactivity||Hu, Ms|
|Tested Application||IHC-P, WB|
|Target Name||Fibrillin 1|
|Immunogen||Synthetic peptide between aa. 2780-2860 of Human Fibrillin 1.|
|Alternate Names||ECTOL1; MFS1; WMS; SGS; SSKS; MASS; GPHYSD2; WMS2; ACMICD; OCTD; Fibrillin-1; FBN|
|Application Note||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.
|Purification||Affinity purification with immunogen.|
|Buffer||PBS, 0.02% Sodium azide, 50% Glycerol and 0.5% BSA.|
|Preservative||0.02% Sodium azide|
|Stabilizer||50% Glycerol and 0.5% BSA|
|Storage Instruction||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.|
|Note||For laboratory research only, not for drug, diagnostic or other use.|
|Gene Full Name||fibrillin 1|
|Background||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]|
|Function||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]|
|Cellular Localization||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]|
|Calculated MW||312 kDa|
|PTM||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]
Images (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.