产品名称
Fibulin-4 Rabbit Polyclonal Antibody
别名
EFEMP2; FBLN4; EGF-containing fibulin-like extracellular matrix protein 2; Fibulin-4; FIBL-4; Protein UPH1
蛋白名称
EGF-containing fibulin-like extracellular matrix protein 2
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=30008
Human Swissprot No.
O95967
Human Swissprot Link
http://www.uniprot.org/uniprotkb/O95967/entry
Mouse Swissprot No.
Q9WVJ9
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9WVJ9
免疫原
The antiserum was produced against synthesized peptide derived from human EFEMP2. AA range:91-140
特异性
Fibulin-4 Polyclonal Antibody detects endogenous levels of Fibulin-4 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
A large number of extracellular matrix proteins have been found to contain variations of the epidermal growth factor (EGF) domain and have been implicated in functions as diverse as blood coagulation, activation of complement and determination of cell fate during development. The protein encoded by this gene contains four EGF2 domains and six calcium-binding EGF2 domains. This gene is necessary for elastic fiber formation and connective tissue development. Defects in this gene are cause of an autosomal recessive cutis laxa syndrome. Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Jan 2011],
组织表达
Brain,Melanoma,Placenta,Synovial membrane t
细胞定位
Secreted, extracellular space, extracellular matrix . Secreted, extracellular space, extracellular matrix, basement membrane . Localizes on the microfibrils surrounding ELN cores. .
功能
disease:Defects in EFEMP2 are a cause of autosomal recessive cutis laxa type I (CL type I) [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. CL type I 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.,similarity:Contains 6 EGF-like domains.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
