ARG21971
anti-Fibrillin 1 antibody [11C1.3] (Biotin)
anti-Fibrillin 1 antibody [11C1.3] (Biotin) for Electron microscopy,ICC/IF,IHC-Frozen sections,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Bovine
Overview
Product Description | Biotin-conjugated Mouse Monoclonal antibody [11C1.3] recognizes Fibrillin 1 |
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Tested Reactivity | Hu, Bov |
Tested Application | EM, ICC/IF, IHC-Fr, IHC-P, WB |
Specificity | Human/Bovine/Japanese Macaque fibrillin-1 |
Host | Mouse |
Clonality | Monoclonal |
Clone | 11C1.3 |
Isotype | IgG1, kappa |
Target Name | Fibrillin 1 |
Antigen Species | Bovine |
Immunogen | Microfibrils from the zonular apparatus of Bovine eye |
Conjugation | Biotin |
Alternate Names | ECTOL1; MFS1; WMS; SGS; SSKS; MASS; GPHYSD2; WMS2; ACMICD; OCTD; Fibrillin-1; FBN |
Application Instructions
Application Suggestion |
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Application Note | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
Properties
Form | Liquid |
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Buffer | PBS and 0.1% Sodium azide. |
Preservative | 0.1% Sodium azide |
Concentration | 0.2 mg/ml |
Storage Instruction | Aliquot and store in the dark at 2-8°C. Keep protected from prolonged exposure to light. 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. |
Bioinformation
Database Links | |
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Gene Symbol | FBN1 |
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] |
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. |