Recombinant human ABCD1/ALD protein (Active) (ab235011)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Active: Yes
- Tags: His tag C-Terminus
- Suitable for: Functional Studies, SDS-PAGE
Description
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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.
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Purity
> 90 % SDS-PAGE. -
Expression system
Escherichia coli -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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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
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Associated products
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Related Products
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.
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Applications
Functional Studies
SDS-PAGE
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Form
Liquid -
Additional notes
Produced in an in vitro E.coli expression system.
Previously labelled as ABCD1.
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Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.2
Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)This product is an active protein and may elicit a biological response in vivo, handle with caution.
General Info
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Alternative names
- ABC42
- Abcd1
- ABCD1_HUMAN
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
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab235011 has not yet been referenced specifically in any publications.