中文名稱    纖連蛋白4抗體    

英文名稱    EFEMP2  

別    名    Fibulin 4; EFEMP2; EGF containing fibulin like extracellular matrix protein 2; EGF-containing fibulin-like extracellular matrix protein 2; FBLN 4; FBLN4; FBLN4_HUMAN; FIBL 4; FIBL-4; FIBL4; Fibulin4; Fibulin-4; MBP 1; MBP1; Mutant p53 binding protein 1; Protein UPH1; UPH 1; UPH1; UPH1 protein.    

供 應 商    遠慕生物
    

研究領域    細胞生物  信號轉導  細胞外基質      

抗體來源    Rabbit    

克隆類型    Polyclonal    

交叉反應    Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,     

產品應用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 纖連蛋白4抗體（石蠟切片需做抗原修復）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    47kDa    

細胞定位    分泌型蛋白     

性    狀    Lyophilized or Liquid    

濃    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human EFEMP2/Fibulin 4    

亞    型    IgG    

純化方法    affinity purified by Protein A    

儲 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存條件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

纖連蛋白4抗體產品介紹    background:

Fibulin proteins contribute to normal development of elastic fiber systems in various types of organs that require elasticity, such as vasculature, lung and skin. Fibulin-4, also known as EFEMP2 (EGF-containing fibulin-like extracellular matrix protein 2), MBP1 or UPH1 is a 443 amino acid secreted protein that contains six EGF-like calcium-binding domains and belongs to the fibulin family. Expressed ubiquitously with highest expression in heart, Fibulin-4 is essential for connective tissue development and elastic fiber formation, and may also play an important role in vascular patterning and collagen biosynthesis. Defects in the gene encoding Fibulin-4 are associated with autosomal recessive cutis laxa type I (CL type I), a connective tissue disorder that is inherited in both an autosomal dominant and an autosomal recessive manner and is characterized by inelastic tissue in all affected areas of the body.

Subcellular Location:
Secreted.

DISEASE:
Defects in EFEMP2 are a cause of cutis laxa autosomal recessive type 1 (ARCL1) [MIM:219100]. Hereditary cutis laxa refers to a heterogeneous group of connective tissue disorders characterized by cutaneous abnormalities and variable systemic manifestations. The most constant clinical feature is loose skin, sagging over the face and trunk. Hereditary cutis laxa is inherited in both autosomal dominant and autosomal recessive modes. ARCL1 shows the most severe phenotype and has the poorest prognosis. In addition to the skin, internal organs enriched in elastic fibers, such as the lung and arteries, are affected

Similarity:
Belongs to the fibulin family.
Contains 6 EGF-like domains.

Database links:

Entrez Gene: 30008 Human

Omim: 604633 Human

SwissProt: O95967 Human

Unigene: 170622 Human


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.