SAB4500863
Anti-Fibrillin-1 antibody produced in rabbit
affinity isolated antibody
Sinónimos:
FBN, M350 kda glycoprotein component extracellular microfibril, MASS, MFS1, fibrillin-1
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About This Item
Productos recomendados
origen biológico
rabbit
conjugado
unconjugated
forma del anticuerpo
affinity isolated antibody
tipo de anticuerpo
primary antibodies
clon
polyclonal
formulario
buffered aqueous solution
mol peso
antigen 312 kDa
reactividad de especies
human, rat, mouse
concentración
~1 mg/mL
técnicas
ELISA: 1:40000
immunohistochemistry: 1:50-1:100
Nº de acceso NCBI
Nº de acceso UniProt
Condiciones de envío
wet ice
temp. de almacenamiento
−20°C
modificación del objetivo postraduccional
unmodified
Información sobre el gen
human ... FBN1(2200)
Categorías relacionadas
Descripción general
Fibrillin-1 is a multidomain cysteine-rich glycoprotein that belongs to the fibrillin protein family. It is encoded by the FBN1 gene mapped to human chromosome 15q21. Fibrillin-1 structure includes 43 calcium-binding epidermal growth factor (cbEGF)–like domains and 78 cysteine-containing TB motifs. The protein is ubiquitously present and is a vital component of elastic fiber–associated microfibrils in connective tissues. Anti-Fibrillin-1 Antibody detects endogenous levels of total Fibrillin-1 protein.
Inmunógeno
The antiserum was produced against synthesized peptide derived from human Fibrillin-1.
Immunogen Range: 2811-2860
Immunogen Range: 2811-2860
Aplicación
Anti-Fibrillin-1 antibody produced in rabbit has been used in immunohistochemical staining (1:50).
Acciones bioquímicas o fisiológicas
Fibrillin-1 protein plays an important role in the fibrillogenesis in elastic tissues and non-elastic tissues. It acts as an anchoring fiber and induces Smad2 signaling by regulating the bioavailability of endogenous transforming growth factor β1 (TGF-β1). Mutation in the FBN1 gene leads to the development of heritable disorder Marfan syndrome. Mutation of this gene also causes thoracic aortic aneurysms in patients without Marfan syndrome.
Características y beneficios
Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.
Forma física
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Cláusula de descargo de responsabilidad
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Código de clase de almacenamiento
10 - Combustible liquids
Clase de riesgo para el agua (WGK)
nwg
Punto de inflamabilidad (°F)
Not applicable
Punto de inflamabilidad (°C)
Not applicable
Certificados de análisis (COA)
Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»
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Los clientes también vieron
Chapter 105 - Heritable Diseases of Connective Tissue
Hawaiian Entomological Society. (2016)
Genetic Basis of Aortic Disease
Hawaiian Entomological Society., 91-100 (2018)
Laure Delhon et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 33(2), 2707-2718 (2018-10-12)
Mutations in the a disintegrin and metalloproteinase with thrombospondin motif-like 2 ( ADAMTSL2) gene are responsible for the autosomal recessive form of geleophysic dysplasia, which is characterized by short stature, short extremities, and skeletal abnormalities. However, the exact function of
H C Dietz et al.
Human molecular genetics, 4 Spec No, 1799-1809 (1995-01-01)
The extracellular microfibril, 10-14 nm in diameter, performs a number of functions, including serving as the scaffolding for deposition of tropoelastin to form elastic fibers. A variety of proteins compose the structure of microfibrils, the most prominent of which are
D M Milewicz et al.
Circulation, 94(11), 2708-2711 (1996-12-01)
Mutations in the FBN1 gene are the cause of the Marfan syndrome, an autosomal dominant disorder with skeletal, ocular, and cardiovascular complications. Aneurysms or dissections of the ascending thoracic aorta are the major cardiovascular complications of the disorder. We tested
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