Anti-Elastin抗体(ab23748)

概述

  • 产品名称Anti-Elastin抗体
    参阅全部 Elastin 一抗
  • 描述
    兔多克隆抗体to Elastin
  • 特异性ab23748 reacts with rat elastin 100%, rat collagen type I, II, III and V <0.1%
  • 经测试应用适用于: ICC/IF, ELISA, RIA, IHC-Pmore details
  • 种属反应性
    与反应: Rat
  • 免疫原

    Purified elastin from rat aorta.

  • 阳性对照
    • Rat skin or aorta

性能

应用

Our Abpromise guarantee covers the use of ab23748 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
ICC/IF Use at an assay dependent concentration.
ELISA Use at an assay dependent concentration.
RIA 1/500.
IHC-P 1/200 - 1/600. Perform enzymatic antigen retrieval before commencing with IHC staining protocol.

靶标

  • 功能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.
  • 组织特异性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.
  • 疾病相关Defects in ELN are a cause of autosomal dominant cutis laxa (ADCL) [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.
    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.
    Note=ELN is located in the Williams-Beuren syndrome (WBS) critical region. WBS results from a hemizygous deletion of several genes on chromosome 7q11.23, thought to arise as a consequence of unequal crossing over between highly homologous low-copy repeat sequences flanking the deleted region. Haploinsufficiency of ELN may be the cause of certain cardiovascular and musculo-skeletal abnormalities observed in the disease.
  • 序列相似性Belongs to the elastin family.
  • 翻译后修饰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.
    Hydroxylation on proline residues within the sequence motif, GXPG, is most likely 4-hydroxy as this fits the requirement for 4-hydroxylation in vertebrates.
  • 细胞定位Secreted > extracellular space > extracellular matrix. Extracellular matrix of elastic fibers.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Elastin antibody
    • ELN antibody
    • ELN_HUMAN antibody
    • SVAS antibody
    • Tropoelastin antibody
    • WBS antibody
    • WS antibody
    see all

Anti-Elastin antibody (ab23748)参考文献

ab23748 has not yet been referenced specifically in any publications.

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