Description

  • Product name

    Recombinant human ABCD1/ALD protein (Active)
  • Biological activity

    Measured by its binding ability in a functional ELISA. Immobilized ab235011 at 5 μg/ml can bind human PEX19, the EC50 of human PEX19 protein is 22.96-33.00 μg/ml.

  • Purity

    > 90 % SDS-PAGE.

  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MPVLSRPRPWRGNTLKRTAVLLALAAYGAHKVYPLVRQCLAPARGLQAPA GEPTQEASGVAAAKAGMNRVFLQRLLWLLRLLFPRVLCRETGLLALHSAA LVSRTFLSVYVARLDGRLARCIVRKDPRAFGWQLLQWLLIALPATFVNSA IRYLEGQLALSFRSRLVAHAYRLYFSQQTYYRVSNMDGRLRNPDQSLTED VVAFAASVAHLYSNLTKPLLDVAVTSYTLLRAARSRGAGTAWPSAIAGLV VFLTANVLRAFSPKFGELVAEEARRKGELRYMHSRVVANSEEIAFYGGHE VELALLQRSYQDLASQINLILLERLWYVMLEQFLMKYVWSASGLLMVAVP IITATGYSESDAEAVKKAALEKKEEELVSERTEAFTIARNLLTAAADAIE RIMSSYKEVTELAGYTARVHEMFQVFEDVQRCHFKRPRELEDAQAGSGTI GRSGVRVEGPLKIRGQVVDVEQGIICENIPIVTPSGEVVVASLNIRVEEG MHLLITGPNGCGKSSLFRILGGLWPTYGGVLYKPPPQRMFYIPQRPYMSV GSLRDQVIYPDSVEDMQRKGYSEQDLEAILDVVHLHHILQREGGWEAMCD WKDVLSGGEKQRIGMARMFYHRPKYALLDECTSAVSIDVEGKIFQAAKDA GIALLSITHRPSLWKYHTHLLQFDGEGGWKFEKLDSAARLSLTEEKQRLE QQLAGIPKMQRRLQELCQILGEAVAPAHVPAPSPQGPGGLQGAST
    • Predicted molecular weight

      85 kDa including tags
    • Amino acids

      1 to 745
    • Tags

      His tag C-Terminus
    • Additional sequence information

      C-terminal 10xHis-tagged

Specifications

Our Abpromise guarantee covers the use of ab235011 in the following tested applications.

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

  • Applications

    Functional Studies

    SDS-PAGE

  • Form

    Liquid
  • Additional notes

    Produced in an in vitro E.coli expression system.

    Previously labelled as ABCD1. 

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    Constituents: Tris buffer, 50% Glycerol

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

General Info

  • Alternative names

    • ABC42
    • Abcd1
    • ABCD1_HUMAN
    • Adrenoleukodystrophy protein
    • ALD
    • Aldgh
    • ALDP
    • AMN
    • ATP binding cassette, sub family D (ALD), member 1
    • ATP-binding cassette sub-family D member 1
    • OTTHUMP00000025960
    • OTTMUSP00000019283
    • RGD1562128
    • RP23 373N8.2
    • X linked adrenoleukodystrophy (ALD) gene homolog
    see all
  • Function

    Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
  • Involvement in disease

    Defects in ABCD1 are the cause of adrenoleukodystrophy X-linked (X-ALD) [MIM:300100]. X-ALD is a peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and 'Addison disease only' (ADO) phenotype.
    Note=The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31.
  • Sequence similarities

    Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.
    Contains 1 ABC transmembrane type-1 domain.
    Contains 1 ABC transporter domain.
  • Cellular localization

    Peroxisome membrane.
  • Information by UniProt

Images

  • ab235011 analyzed by (Tris-Glycine gel) discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

  • Immobilized ab235011 at 5 μg/ml can bind human PEX19, the EC50 of human PEX19 is 22.96-33.00 μg/ml.

References

ab235011 has not yet been referenced specifically in any publications.

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