biological source
mouse
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
689, monoclonal
species reactivity
mouse
species reactivity (predicted by homology)
chicken (based on 100% sequence homology), human (based on 100% sequence homology)
technique(s)
ELISA: suitable, immunohistochemistry: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
wet ice
target post-translational modification
unmodified
Quality Level
Gene Information
human ... EFEMP1(2202)
General description
Fibrillins are glycosolated and disulfide-rich components of the extracellular matrix belonging to the Fibrillin-LTBP protein family. They serve an important function in both nonelastic and elastic tissues such as eye, bone and blood vessels. The functional units of Fibrillin are known as microfibrils, multiprotein assemblies, which act as structural scaffolding for the limiting of the tissue elasticity, structural integrity of different organ systems, the assembly of elastic fibers, and regulation of BMP growth factor signaling. There are three known isoforms; Fibrillin 1, Fibrillin 2, and Fibrillin 3. Fibrillin 3 is the most recently described member of the Fibrillin family, with highest expression in the brain. Alterations in the microfibril assembly often occur in patients with fibrillinopathies such as; Congenital Contractual Arachnodactyly (CCA) which results in disruption of Fibrillin 2, and Marfan’s syndrome resulting from Fibrillin 1 mutation.
~ 350 kDa calculated
Immunogen
Recombinant protein corresponding to human Fibrillin 3.
Application
Anti-Fibrillin (Pan) Antibody, clone 689 detects level of Fibrillin (Pan) & has been published & validated for use in IH, ELISA.
ELISA Analysis: This antibody has been used by an independent laboratory in ELISA (Corson, 2003).
Biochem/physiol Actions
The antibody recognizes isoforms 1, 2, and 3 of fibrillin.
Physical form
Format: Purified
Analysis Note
Control
Mouse skin tissue
Mouse skin tissue
Evaluated by Immunohistochemistry in fresh frozen mouse skin tissue.
Immunohistochemistry Analysis: 1:200 dilution of the antibody detected Fibrillin (Pan) in fresh frozen mouse skin tissue.
Immunohistochemistry Analysis: 1:200 dilution of the antibody detected Fibrillin (Pan) in fresh frozen mouse skin tissue.
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
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存储类别
12 - Non Combustible Liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
Yong Yuan et al.
PloS one, 14(3), e0212569-e0212569 (2019-03-07)
Pseudoexfoliation (PEX) syndrome is an age-related systemic disease with ocular manifestations. The development of animal models is critical in order to elucidate the cause of the disease and to test potential treatment regimens. The purpose of this study is to
Tomoko Matsuzaki et al.
Journal of cellular physiology, 236(3), 1980-1995 (2020-07-31)
Fibrillins (FBNs) form mesh-like structures of microfibrils in various elastic tissues. RECK and FBN1 are co-expressed in many human tissues, suggesting a functional relationship. We found that dermal FBN1 fibers show atypical morphology in mice with reduced RECK expression (RECK-Hypo
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