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Anti-ELN Antibody

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产品名称: Anti-ELN Antibody
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简单介绍

Anti-ELN Antibody


Anti-ELN Antibody  的详细介绍
Name: Anti-ELN Antibody
See all ELN primary antibodies
Description: Rabbit polyclonal antibody to ELN
Specificity: The antibody detects endogenous levels of total ELN protein.
Applications: WB, IHC
Reactivity: Human
Immunogen: Fusion protein corresponding to a region derived from internal residues of human elastin
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Antigen affinity purification.
Concentration: 2.1mg / ml
Formulation: Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Storage: Store at -20?C
Function: Major structural protein of tissues such as aorta and nuchal ligament, which must expand rapidly and recover completely. Molecular determinant of the late arterial morphogenesis, stabilizing arterial structure by regulating proliferation and organization of vascular smooth muscle (By similarity).
Tissue Specificity: 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.
Involvement in Disease: Cutis laxa, autosomal dominant, 1: A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema.

Supravalvular aortic stenosis: Congenital narrowing of the ascending aorta which can occur sporadically, as an autosomal dominant condition, or as one component of Williams-Beuren syndrome.
Sequence Similarities: Belongs to the elastin family.
Post-Translational Modification: Elastin is formed through the cross-linking of its soluble precursor tropoelastin. Cross-linking is initiated through the action of lysyl oxidase on exposed lysines to form allysine. Subsequent spontaneous condensation reactions with other allysine or unmodified lysine residues result in various bi-, tri-, and tetrafunctional cross-links. The most abundant cross-links in mature elastin fibers are lysinonorleucine, allysine aldol, desmosine, and isodesmosine.
Cellular Location: Secreted > Extracellular space > Extracellular matrix.

Extracellular matrix of elastic fibers.
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