Natural Cow Elastin protein (FITC) (ab123533)

製品の概要

製品の詳細

  • 由来Natural
  • 由来Native
  • アミノ酸配列
    • アクセッション番号P04985
    • 生物種Cow
    • 分子量61 kDa
    • 領域27 to 747
    • 配列の追加情報Source = bovine neck ligament elastin
  • 標識FITC. Ex: 493nm, Em: 528nm

関連製品

特性

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.

  • 製品の状態Lyophilised
  • 備考Protect from light.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

関連情報

  • 別名
    • Elastin
    • ELN
    • ELN_HUMAN
    • SVAS
    • Tropoelastin
    • WBS
    • WS
    see all
  • 機能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.
  • 組織特異性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.
  • 関連疾患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.
  • 配列類似性Belongs to the elastin family.
  • 翻訳後修飾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.
  • 細胞内局在Secreted > extracellular space > extracellular matrix. Extracellular matrix of elastic fibers.
  • Information by UniProt

Natural Cow Elastin protein (FITC) (ab123533) 使用論文

ab123533 has not yet been referenced specifically in any publications.

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