Natural牛Elastin protein (FITC) (ab123533)

概述

描述

  • 性质Natural
  • 来源Native
  • 氨基酸序列
    • AccessionP04985
    • 种属Cow
    • 分子量61 kDa
    • 氨基酸27 to 747
    • 额外的序列信息Source = bovine neck ligament elastin
  • 偶联物FITC. Ex: 493nm, Em: 528nm

相关产品

技术指标

Our Abpromise guarantee covers the use of ab123533 in the following tested applications.

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

  • 形式Lyophilised
  • 补充说明Protect from light.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Store at -20°C. Store under Desiccating conditions.

常规信息

  • 别名
    • Elastin
    • ELN
    • ELN_HUMAN
    • SVAS
    • Tropoelastin
    • WBS
    • WS
    see all
  • 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

Natural Cow Elastin protein (FITC) (ab123533)参考文献

ab123533 has not yet been referenced specifically in any publications.

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