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

Description

  • Product name

    Recombinant Human ATP-binding cassette sub-family A member 3 protein
  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Protein fragment
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Predicted molecular weight

      20 kDa
    • Amino acids

      38 to 209

Specifications

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.

  • Applications

    SDS-PAGE

  • Form

    Lyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C.

    Constituents: 0.32% Tris HCl, 0.58% Sodium chloride

  • Reconstitution
    Reconstitute with water to desired concentration.

General Info

  • Alternative names

    • 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
  • Function

    Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol.
  • Tissue specificity

    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.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the ABC transporter superfamily. ABCA family.
    Contains 2 ABC transporter domains.
  • Domain

    Multifunctional polypeptide with two homologous halves, each containing an hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.
  • Cellular localization

    Membrane.
  • Information by UniProt

References

ab125999 has not yet been referenced specifically in any publications.

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