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MAB2499 Anti-Fibrillin-1 Antibody, CT, clone 69

MAB2499
100 µg  
Purchase on Sigma-Aldrich

Ofertas especiales

Descripción

Replacement Information

Ofertas especiales

Tabla espec. clave

Species ReactivityKey ApplicationsHostFormatAntibody Type
HELISA, IP, WB, ICCMPurifiedMonoclonal Antibody
Description
Catalogue NumberMAB2499
Brand Family Chemicon®
Trade Name
  • Chemicon
DescriptionAnti-Fibrillin-1 Antibody, CT, clone 69
References
Product Information
FormatPurified
HS Code3002 15 90
PresentationPurified immunoglobulin. Liquid at 1 mg/mL in 20 mM phosphate buffer, 250 mM NaCl, pH 7.6, containing 0.1% sodium azide.Note: Sodium azide is toxic. MSDS available upon request.
Quality LevelMQ100
Applications
ApplicationDetect Fibrillin-1 using this Anti-Fibrillin-1 Antibody, C-terminus, clone 69 validated for use in ELISA, IP, WB & IC.
Key Applications
  • ELISA
  • Immunoprecipitation
  • Western Blotting
  • Immunocytochemistry
Application NotesImmunoblotting

Immunofluorescence

Immunoprecipitation

ELISA

Optimal working dilutions must be determined by end user.
Biological Information
ImmunogenHuman Fibrillin-1
EpitopeC-terminus
Clone69
HostMouse
SpecificityMonoclonal antibody MAB2499 recognizes human Fibrillin-1. Epitope mapping studies identify the binding site of this antibody to the C-terminal end of the molecule, between amino acid residues 2093 and 2871. The antibody is reactive with human, chicken, and bovine Fibrillin-1.
IsotypeIgG1
Species Reactivity
  • Human
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Entrez Gene SummaryThis 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.
Gene Symbol
  • FBN1
  • FBN
  • MASS
  • WMS
  • MFS1
  • OCTD
  • SGS
UniProt Number
UniProt SummaryFUNCTION: SwissProt: P35555 # Fibrillins are structural components of 10-12 nm extracellular 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.
SIZE: 2871 amino acids; 312312 Da
SUBUNIT: Interacts with COL16A1.
SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix.
PTM: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.
DISEASE: SwissProt: P35555 # Defects in FBN1 are a cause of Marfan syndrome (MFS) [MIM:154700]. MFS is an autosomal dominant disorder that affects the skeletal, ocular, and cardiovascular systems. A wide variety of skeletal abnormalities occurs with MFS, including scoliosis, chest wall deformity, tall stature, abnormal joint mobility. Ectopia lentis occurs in up to about 80% of MFS patients and is almost always bilateral. The leading cause of premature death in MFS patients is progressive dilation of the aortic root and ascending aorta, causing aortic incompetence and dissection. The majority of the more than 600 mutations in FBN1 currently known are point mutations, the rest are frameshifts and splice site mutations. Marfan syndrome has been suggested in at least 2 historical figures, Abraham Lincoln and Paganini. & Defects in FBN1 are a cause of isolated ectopia lentis (EL) [MIM:129600]. The symptoms of this autosomal dominant fibrillinopathy overlap with those of Marfan syndrome, with the exclusion of the skeletal and cardiovascular manifestations. & Defects in FBN1 are the cause of autosomal dominant Weill-Marchesani syndrome (WMS) [MIM:608328]. WMS is a rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma. & Defects in FBN1 are a cause of Shprintzen-Goldberg craniosynostosis syndrome (SGS) [MIM:182212]. SGS is one of a group of disorders characterized by typical features of the Marfan syndrome along with premature closure of the sutures of the skull, causing deformities such as oxycephaly and scaphocephaly. & Defects in FBN1 are a cause of MASS syndrome [MIM:604308]. MASS syndrome is a heritable disorder of connective tissue characterized by involvement of the mitral valve, aorta, skeleton, and skin. MASS syndrome is closely resembling both the Marfan syndrome and the Barlow syndrome. However, no dislocation of the lenses or aneurysmal changes occur in the aorta, and the mitral valve prolapse is by no means invariable.
SIMILARITY: SwissProt: P35555 ## Belongs to the fibrillin family. & Contains 47 EGF-like domains. & Contains 7 TGFBP (TGF-beta binding protein) repeats.
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Usage Statement
  • 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.
Storage and Shipping Information
Storage ConditionsMaintain refrigerated at 2-8°C in undiluted aliquots for up to 12 months.
Packaging Information
Material Size100 µg
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Número de referencia GTIN
MAB2499 04053252267086

Documentation

Anti-Fibrillin-1 Antibody, CT, clone 69 Ficha datos de seguridad (MSDS)

Título

Ficha técnica de seguridad del material (MSDS) 

Anti-Fibrillin-1 Antibody, CT, clone 69 Certificados de análisis

CargoNúmero de lote
MOUSE ANTI FIBRILLIN-1 2700995
MOUSE ANTI FIBRILLIN-1 - 3385787 3385787
MOUSE ANTI FIBRILLIN-1 - 3807903 3807903
MOUSE ANTI FIBRILLIN-1 -2528722 2528722
MOUSE ANTI FIBRILLIN-1 -2547188 2547188
MOUSE ANTI FIBRILLIN-1 -2552270 2552270
MOUSE ANTI FIBRILLIN-1 -2567004 2567004
MOUSE ANTI FIBRILLIN-1 -2593734 2593734
MOUSE ANTI FIBRILLIN-1 -2762258 2762258
MOUSE ANTI FIBRILLIN-1 MONOCLONAL ANTIBODY 2919672

Referencias bibliográficas

Visión general referenciasPub Med ID
Fibrillin-1 staining anomalies are associated with increased staining for TGF-beta and elastic fibre degradation; new clues to the pathogenesis of emphysema.
Mieke M J F Koenders,Ronnie G Wismans,Barry Starcher,Ben C J Hamel,Richard P N Dekhuijzen,Toin H van Kuppevelt
The Journal of pathology  218  2009

Mostrar resumen
19373854 19373854
Expression of latent TGF-beta binding proteins and association with TGF-beta 1 and fibrillin-1 following arterial injury.
Sanjay Sinha, Anthony M Heagerty, C Adrian Shuttleworth, Cay M Kielty
Cardiovascular research  53  971-83  2002

Mostrar resumen
11922907 11922907
Fibrillin-1 in human cartilage: developmental expression and formation of special banded fibers.
Keene, D R, et al.
J. Histochem. Cytochem., 45: 1069-82 (1997)  1997

Mostrar resumen
9267468 9267468