Reactive species
Human;Rat;Mouse;
Applications
WB;IHC;IF;ELISA
Antibody type
Polyclonal Antibody
Protein name
EGF-containing fibulin-like extracellular matrix protein 2
Immunogen
The antiserum was produced against synthesized peptide derived from human EFEMP2. AA range:91-140
Specificity
Fibulin-4 Polyclonal Antibody detects endogenous levels of Fibulin-4 protein.
Constitute
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source
Polyclonal, Rabbit,IgG
Dilution rate
Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications.
Purification process
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Other name
EFEMP2; FBLN4; EGF-containing fibulin-like extracellular matrix protein 2; Fibulin-4; FIBL-4; Protein UPH1
Background
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],
Function
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.,
