Rabbit anti-Human, Mouse FBN1 Polyclonal Antibody | anti-FBN1 antibody

FBN1 Rabbit pAb

Cat. Num. AAA4754675

• Gene Names: FBN1; FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
• Reactivity: Human, Mouse
• Applications: ELISA, Immunohistochemistry
• Purity: Affinity purified
• Synonyms: FBN1; Polyclonal Antibody; FBN1 Rabbit pAb; fibrillin 1; FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2; anti-FBN1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Purity/Purification: Affinity purified
• Form/Format: pH7.4 PBS, 0.05% NaN3, 40% Glycerol

• Applicable Applications for anti-FBN1 antibody: ELISA (EIA), Immunohistochemistry-Paraffin (IHC-P)
• Application Notes: ELISA: 1:1000-1:5000; IHC: 1:50-1:200
• Immunogen: Synthetic peptide of human FBN1
• Conjugation: Unconjugated
• Molecular Function: Fibrillin-1: 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 (PubMed:1860873, PubMed:15062093). Fibrillin-1-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. Fibrillin-1 also plays a key role in tissue homeostasis through specific interactions with growth factors, such as the bone morphogenetic proteins (BMPs), growth and differentiation factors (GDFs) and latent transforming growth factor-beta-binding proteins (LTBPs), cell-surface integrins and other extracellular matrix protein and proteoglycan components (PubMed:27026396). 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 (PubMed:24039232). Mediates cell adhesion via its binding to cell surface receptors integrins ITGAV:ITGB3 and ITGA5:ITGB1 (PubMed:12807887, PubMed:17158881). Binds heparin and this interaction has an important role in the assembly of microfibrils (PubMed:11461921).
• Modification: Unmodified
• Preparation and Storage: Store at -20 degree C. Stable for 12 months from date of receipt.

Immunohistochemistry (IHC)

(The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using 160327(FBN1 Antibody) at dilution 1/47. (Original magnification: ×200))

Related Product Information for anti-FBN1 antibody

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.

Product Categories/Family for anti-FBN1 antibody

Primary antibody

NCBI and Uniprot Product Information

• NCBI GI #: 281485550
• NCBI GeneID: 2200
• NCBI Accession #: NP_000129.3
• NCBI GenBank Nucleotide #: NM_000138.4
• UniProt Accession #: P35555; Q15972; Q75N87; B2RUU0; D2JYH6
• 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; WMS2; ACMICD; ECTOL1; GPHYSD2
• 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, Jul 2008]

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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 15q21.1

Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix; microfibril; extracellular region; 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; metanephros development; skeletal development

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