Recombinant人ATP-binding cassette sub-family A member 3 protein (ab125999)

概述

  • 产品名称
    Recombinant人ATP-binding cassette sub-family A member 3 protein
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Escherichia coli
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 分子量
      20 kDa
    • 氨基酸
      38 to 209

技术指标

Our Abpromise guarantee covers the use of ab125999 in the following tested applications.

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

  • 应用

    SDS-PAGE

  • 形式
    Lyophilised
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Store at -20°C.

    Constituents: 0.32% Tris HCl, 0.58% Sodium chloride

  • 复溶
    Reconstitute with water to desired concentration.

常规信息

  • 别名
    • ABC 3
    • ABC C
    • ABC C transporter
    • ABC transporter 3
    • ABC-C transporter
    • ABC3
    • ABCA 3
    • Abca3
    • ABCA3 protein
    • ABCA3_HUMAN
    • ABCC
    • ATP binding cassette 3
    • ATP binding cassette sub family A (ABC1) member 3
    • ATP binding cassette sub family A member 3
    • ATP binding cassette transporter 3
    • ATP-binding cassette 3
    • ATP-binding cassette sub-family A member 3
    • ATP-binding cassette transporter 3
    • CED7. C. elegans, homolog of
    • EST111653
    • LBM 180
    • LBM180
    • MGC72201
    • P180 Lamellar Body Protein
    • SMDP3
    see all
  • 功能
    Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol.
  • 组织特异性
    Highly expressed in lung, followed by brain, pancreas, skeletal muscle and heart. Weakly expressed in placenta, kidney and liver. Also expressed in medullary thyroid carcinoma cells (MTC) and in C-cell carcinoma.
  • 疾病相关
    Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921]; also called pulmonary alveolar proteinosis due to ABCA3 deficiency. A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.
  • 序列相似性
    Belongs to the ABC transporter superfamily. ABCA family.
    Contains 2 ABC transporter domains.
  • 结构域
    Multifunctional polypeptide with two homologous halves, each containing an hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.
  • 细胞定位
    Membrane.
  • Information by UniProt

Recombinant Human ATP-binding cassette sub-family A member 3 protein (ab125999)参考文献

ab125999 has not yet been referenced specifically in any publications.

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