catalog number :
MBS8242354
products full name :
Anti-Fibrillin 1 Antibody
products short name :
[Fibrillin 1]
products name syn :
[FBN; Fibrillin-1]
other names :
[Fibrillin-1; Fibrillin-1; fibrillin-1; fibrillin 1]
products gene name :
[FBN1]
other gene names :
[FBN1; FBN1; FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2; FBN]
uniprot entry name :
FBN1_HUMAN
reactivity :
Human, Mouse, Rat, Bovine, Pig
specificity :
Recognizes endogenous levels of Fibrillin 1 protein.
purity :
The antibody was purified by immunogen affinity chromatography.
form :
Liquid in 0.42% Potassium Phosphate, 0.87% Sodium Chloride, pH 7.3, 30% Glycerol, and 0.01% Sodium Azide.
storage stability :
Shipped and store at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles
tested application :
Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry (ICC)
app notes :
Western Blot: (1/500 - 1/1000); Immunofluorescence/Immunocytochemistry: (1/100 - 1/500)
image1 heading :
Western Blot (WB)
image2 heading :
Immunofluorescence (IF)
other info1 :
Source: Rabbit. Immunogen: KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of human Fibrillin 1. The exact sequence is proprietary.
products description :
Rabbit polyclonal antibody to Fibrillin 1
ncbi pathways :
Elastic Fibre Formation Pathway (1270251); Extracellular Matrix Organization Pathway (1270244); Integrin Cell Surface Interactions Pathway (1270260)
ncbi summary :
This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate a mature 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. Mutations in this gene are associated with Marfan syndrome and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, and Shprintzen-Goldberg syndrome. [provided by RefSeq, Oct 2015]
uniprot summary :
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: Secreted, signal peptide; Secreted; Extracellular matrix. 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; integrin binding; protein binding; protein complex binding. Biological Process: camera-type eye development; embryonic eye morphogenesis; extracellular matrix disassembly; extracellular matrix organization and biogenesis; heart development; metanephros development; post-embryonic eye morphogenesis; skeletal development. Disease: Acromicric Dysplasia; Ectopia Lentis 1, Isolated, Autosomal Dominant; Geleophysic Dysplasia 2; Marfan Syndrome; Mass Syndrome; Stiff Skin Syndrome; Weill-marchesani Syndrome 2