Rabbit FBN1 Polyclonal Antibody | anti-FBN1 antibody

FBN1 (N2843) Polyclonal Antibody

Cat. Num. AAA3000521

• Gene Names: FBN1; FBN; SGS; WMS; MASS; MFLS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
• Reactivity: Human, Mouse, Rat
• Applications: Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: FBN1; Polyclonal Antibody; FBN1 (N2843) Polyclonal Antibody; Fibrillin-1; Fibrillin1; FBN; anti-FBN1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Fibrillin-1 (N2843) polyclonal antibody detects endogenous levels of Fibrillin-1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 2871

• Applicable Applications for anti-FBN1 antibody: Immunohistochemistry (IHC)
• Application Notes: IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids C-terminus of Human FBN1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) analyzes of FBN1 (N2843) pAb in paraffin-embedded human breast carcinoma tissue at 1:100.)

Related Product Information for anti-FBN1 antibody

The fibrillin family of proteins, including fibrillin-1 (FBN1) and fibrillin-2 (FBN2), are integral components of a distinct subset of extracellular microfibrils. Microfibrils are found in elastic tissues where they facilitate elastic fiber formation and in nonelastic tissue where they support the association of the epithelial cells with the interstitial matrix. Characteristic of the fibrillin proteins are the epidermal growth factor (EGF)-like motifs, which contain a consensus sequence for calcium binding. This calcium association may be critical for protein-protein interactions and stabilization of the microfibrils. Mutations of the FBN1 gene have been shown to result in Marfan syndrome, a disease characterized by abnormal synthesis, secretion and matrix deposition of fibrillin. FBN2 is also linked to a rare, yet similiar, skeletal disorder, congenital contractural arachnodactyly.

NCBI and Uniprot Product Information

• NCBI GI #: 281485550
• NCBI GeneID: 2200
• NCBI Accession #: NP_000129.3
• NCBI GenBank Nucleotide #: NP_000129.3
• UniProt Accession #: P35555
• Molecular Weight: ~ 312kDa
• NCBI Official Full Name: fibrillin-1 preproprotein
• NCBI Official Synonym Full Names: fibrillin 1
• NCBI Official Symbol: FBN1
• NCBI Official Synonym Symbols: FBN; SGS; WMS; MASS; MFLS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
• NCBI Protein Information: fibrillin-1
• 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 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 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