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ELN Polyclonal Antibody
ELN Polyclonal Antibody
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ELN Polyclonal Antibody
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经销商客户: ¥214.5
实验室客户: ¥292.5
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商品描述

商品属性

Target:ELN

Fields:Protein digestion and absorption

Gene Name:ELN

Protein Name:Elastin (Tropoelastin)

Human Gene Id:2006

Human Swiss Prot No:P15502

Mouse Swiss Prot No:P54320

Rat Swiss Prot No:Q99372

Immunogen:Synthesized peptide derived from part region of human protein

Specificity:ELN Polyclonal Antibody detects endogenous levels of protein.

Formulation:Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source:Polyclonal, Rabbit,IgG

Dilution:WB 1:500-2000 ELISA 1:5000-20000

Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration:1 mg/ml

Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD):68kD

Background: This gene encodes a protein that is one of the two components of elastic fibers. The encoded protein is rich in hydrophobic amino acids such as glycine and proline, which form mobile hydrophobic regions bounded by crosslinks between lysine residues. Deletions and mutations in this gene are associated with supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008],

Function:alternative products:Additional isoforms seem to exist,disease:Defects in ELN are a cause of autosomal dominant cutis laxa [MIM:123700]. Cutis laxa is a rare connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. The skin changes are often accompanied by extracutaneous manifestations, including pulmonary emphysema, bladder diverticula, pulmonary artery stenosis and pyloric stenosis.,disease:Defects in ELN are the cause of supravalvular aortic stenosis (SVAS) [MIM:185500]. SVAS is a congenital narrowing of the ascending aorta which can occur sporadically, as an autosomal dominant condition, or as one component of Williams-Beuren syndrome.,disease:Haploinsufficiency of ELN may be the cause of certain cardiovascular and musculo-skeletal abnormalities observed in Williams-Beuren syndrome (WBS) [

Subcellular Location:Secreted, extracellular space, extracellular matrix . Extracellular matrix of elastic fibers. .

Expression:Expressed within the outer myometrial smooth muscle and throughout the arteriolar tree of uterus (at protein level). Also expressed in the large arteries, lung and skin.

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