Antibody Name:	Anti-FBN1 Antibody
Antibody SKU:	CAB16677
Antibody Size:	20uL, 50uL, 100uL
Application:	IF
Reactivity:	Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 360-460 of human FBN1 (NP_000129.3).

Application:	IF
Recommended Dilution:	IF 1:50 - 1:200
Reactivity:	Mouse, Rat
Positive Samples:

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 360-460 of human FBN1 (NP_000129.3).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	CDAG RCWS PGVT VAPE MCPI RATE DFNK LCSV PMVI PGRP EYPP PPLG PIPP VLPV PPGF PPGP QIPV PRPP VEYL YPSR EPPR VLPV NVTD YCQL VRYL C
Gene ID:	2200
Uniprot:	P35555
Cellular Location:
Calculated MW:	312kDa
Observed MW:	Refer to figures

Synonyms:

FBN1, ACMICD, ECTOL1, FBN, GPHYSD2, MASS, MFLS, MFS1, OCTD, SGS, SSKS, WMS, WMS2

Background:

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Extracellular matrix; Secreted; Secreted, signal peptide Chromosomal Location of Human Ortholog: 15q21.1 Cellular Component: basement membrane; 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; integrin binding; protein binding; protein complex binding Biological Process: camera-type eye development; cell adhesion mediated by integrin; embryonic eye morphogenesis; extracellular matrix disassembly; extracellular matrix organization and biogenesis; heart development; negative regulation of osteoclast differentiation; post-embryonic eye morphogenesis; skeletal 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
NCBI Summary:	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 Code:	P35555
NCBI GenInfo Identifier:	311033452
NCBI Gene ID:	2200
NCBI Accession:	P35555.3
UniProt Secondary Accession:	P35555,Q15972, Q75N87, B2RUU0, D2JYH6,
UniProt Related Accession:	P35555
Molecular Weight:	312,237 Da
NCBI Full Name:	Fibrillin-1
NCBI 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

	Immunofluorescence analysis of C6 cells using FBN1 Polyclonal Antibody (CAB16677) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
	Immunofluorescence analysis of NIH-3T3 cells using FBN1 Polyclonal Antibody (CAB16677) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.