ABCD1 Protein, Human (Cell-Free, His)

ABCD1, Human (His) is one of the four ATP-binding cassette (ABC) proteins in subfamily D. Dysfunction of ABCD1 causes neurodegenerative disorder X-linked adrenoleukodystrophy (X-ALD).

ALDP contains six putative transmembrane domains (TMDs) and a nucleotide-binding domain (NBD). ALDP is synthesized on free polysomes, post-translationally transported to peroxisomes, and inserted into the membranes. During this process, ALDP interacts with Pex19p, a chaperone-like protein for intracellular trafficking of PMP, the complex is targeted to Pex3p on the peroxisomal membranes and then ALDP is inserted into the membranes. After integration into the membranes, ALDP is thought to form mainly a homodimer and in part a heterodimer with other peroxisomal ABC proteins such as 70-kDa peroxisomal membrane protein (PMP70/ABCD3) and adrenoleukodystrophy-related protein (ALDRP/ABCD2). Dysfunction of ABCD1 causes neurodegenerative disorder X-linked adrenoleukodystrophy (X-ALD), which is characterized by the abnormal accumulation of VLCFA due to impaired peroxisomal β-oxidation^[1][2].

Human

E. coli Cell-free

C-10*His

P33897 (M1-T745)

215  [NCBI]

MPVLSRPRPWRGNTLKRTAVLLALAAYGAHKVYPLVRQCLAPARGLQAPAGEPTQEASGVAAAKAGMNRVFLQRLLWLLRLLFPRVLCRETGLLALHSAALVSRTFLSVYVARLDGRLARCIVRKDPRAFGWQLLQWLLIALPATFVNSAIRYLEGQLALSFRSRLVAHAYRLYFSQQTYYRVSNMDGRLRNPDQSLTEDVVAFAASVAHLYSNLTKPLLDVAVTSYTLLRAARSRGAGTAWPSAIAGLVVFLTANVLRAFSPKFGELVAEEARRKGELRYMHSRVVANSEEIAFYGGHEVELALLQRSYQDLASQINLILLERLWYVMLEQFLMKYVWSASGLLMVAVPIITATGYSESDAEAVKKAALEKKEEELVSERTEAFTIARNLLTAAADAIERIMSSYKEVTELAGYTARVHEMFQVFEDVQRCHFKRPRELEDAQAGSGTIGRSGVRVEGPLKIRGQVVDVEQGIICENIPIVTPSGEVVVASLNIRVEEGMHLLITGPNGCGKSSLFRILGGLWPTYGGVLYKPPPQRMFYIPQRPYMSVGSLRDQVIYPDSVEDMQRKGYSEQDLEAILDVVHLHHILQREGGWEAMCDWKDVLSGGEKQRIGMARMFYHRPKYALLDECTSAVSIDVEGKIFQAAKDAGIALLSITHRPSLWKYHTHLLQFDGEGGWKFEKLDSAARLSLTEEKQRLEQQLAGIPKMQRRLQELCQILGEAVAPAHVPAPSPQGPGGLQGAST

Approximately 84.9 kDa

Greater than 90% as determined by reducing SDS-PAGE.

<1 EU/μg, determined by LAL method.

• [1]. Takahashi N, et, al. Adrenoleukodystrophy: subcellular localization and degradation of adrenoleukodystrophy protein (ALDP/ABCD1) with naturally occurring missense mutations. J Neurochem. 2007 Jun;101(6):1632-43.  [Content Brief]

  [2]. Kawaguchi K, et, al. Acyl-CoA thioesterase activity of peroxisomal ABC protein ABCD1 is required for the transport of very long-chain acyl-CoA into peroxisomes. Sci Rep. 2021 Jan 26;11(1):2192.  [Content Brief]