Western Blot (WB)
(Western Blot: Sample: Recombinant FBN1, Human.)
Rabbit anti-Human Fibrillin 1 Polyclonal Antibody | anti-FBN1 antibody
Polyclonal Antibody to Fibrillin 1 (FBN1)
MSPILGYWKI KGLVQPTRLL LEYLEEKYEE HLYERDEGDK WRNKKFELGL EFPNLPYYID GDVKLTQSMA IIRYIADKHN MLGGCPKERA EISMLEGAVL DIRYGVSRIA YSKDFETLKV DFLSKLPEML KMFEDRLCHK TYLNGDHVTH PDFMLYDALD VVLYMDPMCL DAFPKLVCFK KRIEAIPQID KYLKSSKYIA WPLQGWQATF GGGDHPPKSD GSTSGSGHHH HHHSAGLVPR GSTAIGMKET AAAKFERQHM DSPDLGTLEV LFQGPLGS-VT DYCQLVRYLC QNGRCIPTPG SCRCECNKGF QLDLRGECID VDECEKNPCA GGECINNQGS YTCQCRAGYQ STLTRTECRD IDECLQNGRI CNNGRCINTD GSFHCVCNAG FHVTRDGKNC EDMDECSIRN
Immunocytochemistry in formalin fixed cells: 5-20ug/mL
Immunohistochemistry in formalin fixed frozen section: 5-20ug/mL
Immunohistochemistry in paraffin section: 5-20ug/mL
Enzyme-linked Immunosorbent Assay: 0.05-2ug/mL
Optimal working dilutions must be determined by end user.
Western Blot (WB)
(Western Blot: Sample: Recombinant FBN1, Human.)
Western Blot (WB)
(Western Blot: Sample: Recombinant FBN1, Human.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate two proteins including the extracellular matrix component fibrillin-1 and the protein hormone asprosin. Fibrillin-1 is an extracellular matrix glycoprotein that serves as a structural component of calcium-binding microfibrils. These microfibrils provide force-bearing structural support in elastic and nonelastic connective tissue throughout the body. Asprosin, secreted by white adipose tissue, has been shown to regulate glucose homeostasis. Mutations in this gene are associated with Marfan syndrome and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, Shprintzen-Goldberg syndrome and neonatal progeroid syndrome. [provided by RefSeq, Apr 2016]
Uniprot Description
FBN1: a structural component of the 10-12 nm diameter microfibrils of the extracellular matrix, which conveys both structural and regulatory properties to load-bearing connective tissues. FBN1-containing microfibrils provide long-term force bearing structural support. In tissues such as the lung, blood vessels and skin, microfibrils form the periphery of the elastic fiber, acting as a scaffold for the deposition of elastin. In addition, microfibrils can occur as elastin-independent networks in tissues such as the ciliary zonule, tendon, cornea and glomerulus where they provide tensile strength and have anchoring roles. Plays a key role in tissue homeostasis through specific interactions with growth factors, such as the bone morphogenetic proteins (BMPs), growth and differentiation factors and latent transforming growth factor-beta-binding proteins, cell-surface integrins and other extracellular matrix protein and proteoglycan components. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Negatively regulates osteoclastogenesis by binding and sequestering an osteoclast differentiation and activation factor TNFSF11. This leads to disruption of TNFSF11-induced Ca2+ signaling and impairment of TNFSF11-mediated nuclear translocation and activation of transcription factor NFATC1 which regulates genes important for osteoclast differentiation and function. Mediates cell adhesion via its binding to cell surface receptors integrins ITGB3 and ITGB1. Binds heparin and this interaction has an important role in the assembly of microfibrils. Cleavage of N- and C-termini is required for incorporation into the extracellular matrix and assembly into microfibrils. Its cleaved C-terminal peptide (2732 ¿ 2871) is the hormone asprosin, secreted by white adipose tissue that targets the liver, stimulating the release of glucose and increasing plasma glucose levels. Acts in response to fasting, and promotes hepathocyte glucose release via a PKA-dependent pathway.
Protein type: Extracellular matrix; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 15q21.1
Cellular Component: basement membrane; endoplasmic reticulum lumen; extracellular matrix; extracellular region; extracellular space; microfibril; proteinaceous extracellular matrix
Molecular Function: calcium ion binding; extracellular matrix constituent conferring elasticity; extracellular matrix structural constituent; heparin binding; hormone activity; integrin binding; protein binding; protein complex binding
Biological Process: camera-type eye development; cell adhesion mediated by integrin; cellular protein metabolic process; embryonic eye morphogenesis; extracellular matrix disassembly; extracellular matrix organization; heart development; metanephros development; negative regulation of osteoclast differentiation; post-embryonic eye morphogenesis; post-translational protein modification; skeletal system development
Disease: Acromicric Dysplasia; Ectopia Lentis 1, Isolated, Autosomal Dominant; Geleophysic Dysplasia 2; Marfan Lipodystrophy Syndrome; Marfan Syndrome; Mass Syndrome; Stiff Skin Syndrome; Weill-marchesani Syndrome 2
Research Articles on FBN1
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Product Notes
The FBN1 fbn1 (Catalog #AAA2027646) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Polyclonal Antibody to Fibrillin 1 (FBN1) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Fibrillin 1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western blotting: 1-5ug/mL Immunocytochemistry in formalin fixed cells: 5-20ug/mL Immunohistochemistry in formalin fixed frozen section: 5-20ug/mL Immunohistochemistry in paraffin section: 5-20ug/mL Enzyme-linked Immunosorbent Assay: 0.05-2ug/mL Optimal working dilutions must be determined by end user. Researchers should empirically determine the suitability of the FBN1 fbn1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Antigen: The target protein is fused with two N-terminal Tags, His-tag and GST-tag and its sequence is listed below. MSPILGYWKI KGLVQPTRLL LEYLEEKYEE HLYERDEGDK WRNKKFELGL EFPNLPYYID GDVKLTQSMA IIRYIADKHN MLGGCPKERA EISMLEGAVL DIRYGVSRIA YSKDFETLKV DFLSKLPEML KMFEDRLCHK TYLNGDHVTH PDFMLYDALD VVLYMDPMCL DAFPKLVCFK KRIEAIPQID KYLKSSKYIA WPLQGWQATF GGGDHPPKSD GSTSGSGHHH HHHSAGLVPR GSTAIGMKET AAAKFERQHM DSPDLGTLEV LFQGPLGS-V T DYCQLVRYLC QNGRCIPTPG SCRCECNKGF QLDLRGECID VDECEKNPCA GGECINNQGS YTCQCRAGYQ STLTRTECRD IDECLQNGRI CNNGRCINTD GSFHCVCNAG FHVTRDGKNC EDMDECSIRN. It is sometimes possible for the material contained within the vial of "Fibrillin 1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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