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Anti-Fibrillin (Pan), clone 689 (mouse monoclonal)

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货号：MAB2641

品牌：Millipore 密理博

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重要规格表

Species Reactivity Key Applications Host Format Antibody Type
M, H, Ch IHC, ELISA M Purified Monoclonal Antibody
Description
Catalogue Number MAB2641
Description Anti-Fibrillin (Pan) Antibody, clone 689
Alternate Names
  • EGF-containing fibulin-like extracellular matrix protein 1
  • Fibrillin-like protein
  • Extracellular protein S1-5
Background Information 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.
References
Product Information
Format Purified
Control
  • Mouse skin tissue
Presentation Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide.
Applications
Application Anti-Fibrillin (Pan) Antibody, clone 689 detects level of Fibrillin (Pan) & has been published & validated for use in IH, ELISA.
Key Applications
  • Immunohistochemistry
  • ELISA
Application Notes ELISA Analysis: This antibody has been used by an independent laboratory in ELISA (Corson, 2003).
Biological Information
Immunogen Recombinant protein corresponding to human Fibrillin 3.
Clone 689
Concentration Please refer to the Certificate of Analysis for the lot-specific concentration.
Host Mouse
Specificity The antibody recognizes isoforms 1, 2, and 3 of fibrillin.
Isotype IgG1κ
Species Reactivity
  • Mouse
  • Human
  • Chicken
Species Reactivity Note Demonstrated to react with mouse. Predicted to react with human and chicken based on 100% sequence homology.
Antibody Type Monoclonal Antibody
Entrez Gene Number
Entrez Gene Summary This gene encodes a protein that belongs to the fibrillin gene family. Fibrillins are extracellular matrix molecules that assemble into microfibrils in many connective tissues. This gene is most highly expressed in fetal tissues and its protein product is localized to extracellular microfibrils of developing skeletal elements, skin, lung, kidney, and skeletal muscle. This gene is potentially involved in Weill-Marchesani syndrome. While several transcript variants may exist for this gene, their full-length natures have not been described to date. [provided by RefSeq].
Gene Symbol
  • EFEMP1
  • FBNL
  • MLVT
  • FBLN3
  • RAD
  • fibrillin-like
  • Fibulin-3
  • S1-5
  • FIBL-3
  • HRD
  • MTLV
Purification Method Protein G Purified
UniProt Number
UniProt Summary FUNCTION: 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-containing microfibrils provide long-term force bearing structural support.

SUBCELLULAR LOCATION: Secreted › extracellular space › extracellular matrix

TISSUE SPECIFICITY: Predominantly expressed in connective tissues such as skeletal muscle, tendon, skin, perichondrium and periosteum. Highly expressed in fetal lung, brain, kidney. Expressed at low level in prostate, testis, mammary gland, uterus, ovary, placenta, bladder, adrenal gland, thyroid, fetal thymus, fetal liver, liver, fetal heart and heart.

PTM: Probably forms intermolecular disulfide bonds either with other FBN3 molecules or with other components of the microfibrils (by similarity).

SEQUENCE SIMILARITIES: Belongs to the fibrillin family.

Contains 44 EGF-like domains.

Contains 9 TB (TGF-beta binding) domains.

SEQUENCE CAUTION: The sequence BAB47408.2 differs from that shown. Reason: Erroneous initiation.
Molecular Weight ~ 350 kDa calculated
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality Assurance 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.
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 Conditions Stable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size 100 µg
Transport Information
Supplemental Information
Specifications

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