Molecular weight (DA)
55kD
Immunogen
The antiserum was produced against synthesized peptide derived from human EFEMP1. AA range:111-160
Specificity
Fibulin-3 Polyclonal Antibody detects endogenous levels of Fibulin-3 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/10000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes a member of the fibulin family of extracellular matrix glycoproteins. Like all members of this family, the encoded protein contains tandemly repeated epidermal growth factor-like repeats followed by a C-terminus fibulin-type domain. This gene is upregulated in malignant gliomas and may play a role in the aggressive nature of these tumors. Mutations in this gene are associated with Doyne honeycomb retinal dystrophy. Alternatively spliced transcript variants that encode the same protein have been described.[provided by RefSeq, Nov 2009],
Function
alternative products:Experimental confirmation may be lacking for some isoforms,disease:Defects in EFEMP1 are a cause of Doyne honeycomb retinal dystrophy (DHRD) [MIM:126600]; also known as malattia leventinese (MLVT OR ML). DHRD is an autosomal dominant disease characterized by yellow-white deposits known as drusen that accumulate beneath the retinal pigment epithelium.,online information:Retina International's Scientific Newsletter,similarity:Belongs to the fibulin family.,similarity:Contains 6 EGF-like domains.,
Protein name
EGF-containing fibulin-like extracellular matrix protein 1
Other name
EFEMP1; FBLN3; FBNL; EGF-containing fibulin-like extracellular matrix protein 1; Extracellular protein S1-5; Fibrillin-like protein; Fibulin-3; FIBL-3
Human protein sequence Database
Q12805
Mouse protein sequence database
Q8BPB5
Rat protein sequence database
O35568
Cellular localization
Secreted, extracellular space . Secreted, extracellular space, extracellular matrix . Localizes to the lamina propria underneath the olfactory epithelium. .
Tissue expression
In the eye, associated with photoreceptor outer and inner segment regions, the nerve fiber layer, outer nuclear layer and inner and outer plexiform layers of the retina.
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