Rabbit Fibrillin 1, exon 10, Pro-rich Domain Polyclonal Antibody | anti-FBN1 antibody

Fibrillin 1, exon 10, Pro-rich Domain

Cat. Num. AAA616265

• Gene Names: FBN1; FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS
• Reactivity: Bovine, Human
• Applications: ELISA, Western Blot, Immunoprecipitation, Immunohistochemistry, Immunocytochemistry
• Purity: Serum; Serum
• Synonyms: Fibrillin 1; exon 10; Pro-rich Domain; Polyclonal Antibody; Anti -Fibrillin 1; anti-FBN1 antibody

• Host: Rabbit
• Reactivity: Bovine, Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Strongly recognizes human fibrillin-1. Species Crossreactivity: bovine. Does not crossreact with full-length fibrillin-2 secreted by bovine auricular chondroblasts.
• Purity/Purification: Serum; Serum
• Form/Format: Supplied as a liquid in PBS with 40% glycerol.

• Applicable Applications for anti-FBN1 antibody: ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP), Immunohistochemistry (IHC), Immunocytochemistry (ICC)
• Application Notes: Suitable for use in Immunohistochemistry, ELISA, Western Blot, Immunocytochemistry and Immunoprecipitation.; Dilution: ELISA: 1:1000-1:2000; Western Blot: 1:200-1:500; Immunohistochemistry: 1:50-1:100; Immunocytochemistry: 1:50-1:100; Immunoprecipitation: 1:100-1:200
• Immunogen: Protein fragment containing proline-rich domain of exon 10 of human fibrillin-1.
• Note: In many cases, antigenicity of elastic fiber components can be enhanced by treating with 6M guanidine HCl, 50mM dithiothreitol, 20mM Tris pH 8.0 for 15 minutes. After washing with 20mM Tris pH 8.0, the slides are treated with 100mM iodoacetamide in the dark for 15 minutes, washed and stained using normal protocols.
• Preparation and Storage: May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for anti-FBN1 antibody

Fibrillin-1 is an extracellular acidic protein with a high cysteine content and an extended thread-like shape with mosaic composition of different types of extracellular modules. Most of the fibrillin molecule is contributed by 47 epidermal growth factor-like (EGF-like) repeats; 43 of them have a consensus sequence for calcium binding (cb). Other modules including 8-cysteine motifs (seven), hybrid motifs (two), and unique domains (three) are interspersed throughout the molecule. Fibrillin-1 contains a proline-rich region close to the N-terminus that is replaced by a glycine-rich region in fibrillin-2. The fibrillins are found throughout the connective tissue as integral components of extended fibrils. There is an aberrant expression of fibrillin-1 in Marfan syndrome.

Product Categories/Family for anti-FBN1 antibody

Antibodies; Abs to Fibrillin

NCBI and Uniprot Product Information

• NCBI GI #: 251757263
• NCBI GeneID: 2200
• UniProt Accession #: P35555; Q15972; Q75N87; B2RUU0; Q59HB9; Q75N88; Q75N89; Q9NP01
• Molecular Weight: 312,237 Da
• NCBI Official Full Name: Fibrillin-1
• NCBI Official Synonym Full Names: fibrillin 1
• NCBI Official Symbol: FBN1
• NCBI Official Synonym Symbols: FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS
• NCBI Protein Information: fibrillin-1; fibrillin 15
• UniProt Protein Name: Fibrillin-1
• Protein Family: Fibrillin
• UniProt Gene Name: FBN1
• UniProt Synonym Gene Names: FBN
• UniProt Entry Name: FBN1_HUMAN

NCBI Description

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]

Uniprot Description

FBN1: a large, extracellular matrix glycoprotein of the fibrillin family that serves as a structural component of 10-12 nm 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 in elastic and nonelastic connective tissue throughout the body. May regulate osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects in this protein are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. Interacts with COL16A1.

Protein type: Extracellular matrix; Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 15q21.1

Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix; extracellular region; microfibril; basement membrane

Molecular Function: protein binding; extracellular matrix structural constituent; calcium ion binding

Biological Process: extracellular matrix disassembly; extracellular matrix organization and biogenesis; heart development; skeletal development; metanephros development

Disease: Ectopia Lentis 1, Isolated, Autosomal Dominant; Mass Syndrome; Weill-marchesani Syndrome 2; Stiff Skin Syndrome; Acromicric Dysplasia; Marfan Syndrome; Geleophysic Dysplasia 2