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MAB2642 Anti-Fibrillin-2 Antibody, clone 48

MAB2642
100 µg  
Purchase on Sigma-Aldrich

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Kulcsspecifikációk táblázata

Species ReactivityKey ApplicationsHostFormatAntibody Type
HIHC, ELISA, IP, ICCMPurifiedMonoclonal Antibody
Description
Catalogue NumberMAB2642
DescriptionAnti-Fibrillin-2 Antibody, clone 48
Alternate Names
  • fibrillin 2
  • congenital contractural arachnodactyly
  • Fibrillin 5
Background InformationFibrillin-2 is a part of the Fibrillin family of glycoproteins, which are essential factors in the formation and maintenance of elastic fibers known as microfibrils within the extracellular matrix. Various researchers have suggested that Fibrillin-2 has a role in the mediation of microfibril assembly during early elastogenesis. Defects in Fibrillin-2 are causal to a rare disorder involving autosomal dominant connective tissue known as congenital contractual arachnodactyly (CCA), also known as Beals syndrome or distal arthrogryposis type 9 (DA9), and is similar to Marfan syndrome.
References
Product Information
FormatPurified
Control
  • Liver cancer and papillary serous carcinoma tissue
PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-Fibrillin-2 Antibody, clone 48 is an antibody against Fibrillin-2 for use in IH, ELISA, IP & IC.
Key Applications
  • Immunohistochemistry
  • ELISA
  • Immunoprecipitation
  • Immunocytochemistry
Application NotesELISA Analysis: A previous lot was used by an independent laboratory in ELISA (Charbonneau, 2003).

Immunofluorescence Analysis: A previous lot was used by an independent laboratory in IF (Charbonneau, 2003).
Biological Information
ImmunogenRecombinant protein corresponding to human Fibrillin-2.
EpitopeUnknown
Clone48
ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
HostMouse
SpecificityThe antibody recognizes Fibrillin-2.
IsotypeIgG1κ
Species Reactivity
  • Human
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Entrez Gene SummaryThe protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq].
Gene Symbol
  • DA9
  • CCA
  • FBN2
Purification MethodProtein G Purified
UniProt Number
UniProt SummaryFUNCTION: 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-2-containing microfibrils regulate the early process of elastic fiber assembly.

SUBCELLULAR LOCATION: Secreted › extracellular space › extracellular matrix.

INVOLVEMENT IN DISEASE: Defects in FBN2 are the cause of congenital contractural arachnodactyly (CCA) [MIM:121050]; also known as Beals syndrome or distal arthrogryposis type 9 (DA9). CCA is a rare, autosomal dominant connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Phenotypically similar to Marfan syndrome, CCA does not affect the aorta and the eyes.

SEQUENCE SIMILARITIES: Belongs to the fibrillin family.

Contains 47 EGF-like domains.

Contains 9 TB (TGF-beta binding) domains.
Molecular Weight~ 215 kDa
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated by Immunohistochemistry in liver cancer and papillary serous carcinoma paraffin-embedded tissue sections.

Immunohistochemistry Analysis: 1:50 dilution of this antibody detected Fibrillin-2 in paraffin-embedded tissue sections of liver cancer and papillary serous carcinoma.
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 ConditionsStable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size100 µg
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Katalógusszám GTIN
MAB2642 04053252478703

Documentation

Anti-Fibrillin-2 Antibody, clone 48 MSDS

Title

Safety Data Sheet (SDS) 

Anti-Fibrillin-2 Antibody, clone 48 Certificates of Analysis

TitleLot Number
Anti-Fibrillin-2, clone 48 - 2120983 2120983
Anti-Fibrillin-2, clone 48 - 3216026 3216026
Anti-Fibrillin-2, clone 48 - 3697335 3697335
Anti-Fibrillin-2, clone 48 - NG1928924 NG1928924
Anti-Fibrillin-2, clone 48 - NRG1739024 NRG1739024
Anti-Fibrillin-2, clone 48 -2664461 2664461
Anti-Fibrillin-2, clone 48 -2733577 2733577
Anti-Fibrillin-2, clone 48 Monoclonal Antibody 3041055

References

Reference overviewPub Med ID
Fibrillin-2, tenascin-C, matrilin-2, and matrilin-4 are strongly expressed in the epithelium of human granular and lattice type I corneal dystrophies.
Szalai, E; Felszeghy, S; Hegyi, Z; Módis, L; Berta, A; Kaarniranta, K
Molecular vision  18  1927-36  2011

Kivonat megmutatása
22876117 22876117