ARG62490
anti-Fibrillin 1 antibody [11C1.3]
anti-Fibrillin 1 antibody [11C1.3] for Electron microscopy,Flow cytometry,ICC/IF,IHC-Frozen sections,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human
Signaling Transduction antibody
Overview
| Product Description | Mouse Monoclonal antibody [11C1.3] recognizes Fibrillin 1 |
|---|---|
| Tested Reactivity | Hu |
| Tested Application | EM, FACS, ICC/IF, IHC-Fr, IHC-P, WB |
| Host | Mouse |
| Clonality | Monoclonal |
| Clone | 11C1.3 |
| Isotype | IgG1, kappa |
| Target Name | Fibrillin 1 |
| Antigen Species | Bovine |
| Immunogen | Microfibrils from zonular apparatus of bovine eye. |
| Epitope | Amino acids 451 - 909. |
| Conjugation | Un-conjugated |
| Alternate Names | ECTOL1; MFS1; WMS; SGS; SSKS; MASS; GPHYSD2; WMS2; ACMICD; OCTD; Fibrillin-1; FBN |
Application Instructions
| Application Note | WB: 1 - 2 µg/ml FACS: 2µg for 106 cells ICC/IF: 5 µg/ml IHC: 1/10 - 1/500 * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
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Properties
| Form | Liquid |
|---|---|
| Purification | Protein G purified |
| Buffer | 1X PBS buffer with < 0.1% sodium azide. |
| Preservative | < 0.1% sodium azide. |
| Concentration | 2 mg/ml |
| 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. |
Bioinformation
| Database Links | |
|---|---|
| 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 (By similarity). [UniProt] |
| Cellular Localization | Extracellular microfibrils |
| Research Area | Signaling Transduction antibody |
| 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. |
Clone References
Epithelial-mesenchymal status influences how cells deposit fibrillin microfibrils.
ICC/IF / Human
Latent transforming growth factor-β binding protein 2 negatively regulates coalescence of oxytalan fibers induced by stretching stress.
IHC / Human
Discovery of putative oocyte quality markers by comparative ExacTag proteomics.
WB / Pig
Dermal tissue and cellular expression of fibrillin-1 in diffuse cutaneous systemic sclerosis.
WB / Human
Decreased gene expression of fibrillin-1 in stress urinary incontinence.
IHC / Human
Increased expression of extracellular matrix proteins in rapid atrial pacing-induced atrial fibrillation.
IHC / Pig
