Antibody Name:	Anti-ABCD1 Antibody
Antibody SKU:	CAB12141
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 340-500 of human ABCD1 (NP_000024.2).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	LO2, U-87MG, HeLa

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 340-500 of human ABCD1 (NP_000024.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	SASG LLMV AVPI ITAT GYSE SDAE AVKK AALE KKEE ELVS ERTE AFTI ARNL LTAA ADAI ERIM SSYK EVTE LAGY TARV HEMF QVFE DVQR CHFK RPRE LEDA QAGS GTIG RSGV RVEG PLKI RGQV VDVE QGII CENI PIVT PSGE VVVA SLNI RVEE G
Gene ID:	215
Uniprot:	P33897
Cellular Location:	Multi-pass membrane protein, Peroxisome membrane
Calculated MW:	82kDa
Observed MW:	83kDa

Synonyms:

ABCD1, ABC42, ALD, ALDP, AMN

Background:

The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. This peroxisomal membrane protein is likely involved in the peroxisomal transport or catabolism of very long chain fatty acids. Defects in this gene have been identified as the underlying cause of adrenoleukodystrophy, an X-chromosome recessively inherited demyelinating disorder of the nervous system.

UniProt Protein Function:	ABCD1: Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity. Defects in ABCD1 are the cause of adrenoleukodystrophy X- linked (X-ALD). 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. The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31. Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.
UniProt Protein Details:	Protein type:Membrane protein, multi-pass; Hydrolase; Mitochondrial; Membrane protein, integral; Transporter; Transporter, ABC family Chromosomal Location of Human Ortholog: Xq28 Cellular Component: peroxisomal membrane; integral to peroxisomal membrane; membrane; mitochondrion; perinuclear region of cytoplasm; cytoplasm; peroxisome; cytosol Molecular Function:identical protein binding; protein binding; enzyme binding; protein homodimerization activity; peroxisomal fatty acyl CoA transporter activity; transporter activity; ATPase activity, coupled to transmembrane movement of substances; ATPase activity; ATP binding Biological Process: fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid beta-oxidation; peroxisomal membrane transport; peroxisome organization and biogenesis; peroxisomal long-chain fatty acid import; very-long-chain fatty acid catabolic process; unsaturated fatty acid metabolic process; linoleic acid metabolic process; cellular lipid metabolic process; transmembrane transport; long-chain fatty acid catabolic process Disease: Adrenoleukodystrophy
NCBI Summary:	The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. This peroxisomal membrane protein is likely involved in the peroxisomal transport or catabolism of very long chain fatty acids. Defects in this gene have been identified as the underlying cause of adrenoleukodystrophy, an X-chromosome recessively inherited demyelinating disorder of the nervous system. [provided by RefSeq, Jul 2008]
UniProt Code:	P33897
NCBI GenInfo Identifier:	7262393
NCBI Gene ID:	215
NCBI Accession:	NP_000024.2
UniProt Secondary Accession:	P33897,Q6GTZ2,
UniProt Related Accession:	P33897
Molecular Weight:	82,937 Da
NCBI Full Name:	ATP-binding cassette sub-family D member 1
NCBI Synonym Full Names:	ATP-binding cassette, sub-family D (ALD), member 1
NCBI Official Symbol:	ABCD1
NCBI Official Synonym Symbols:	ALD; AMN; ALDP; ABC42
NCBI Protein Information:	ATP-binding cassette sub-family D member 1; adrenoleukodystrophy protein
UniProt Protein Name:	ATP-binding cassette sub-family D member 1
UniProt Synonym Protein Names:	Adrenoleukodystrophy protein
Protein Family:	ABC transporter D family
UniProt Gene Name:	ABCD1
UniProt Entry Name:	ABCD1_HUMAN

	Western blot analysis of extracts of various cell lines, using ABCD1 antibody (CAB12141) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
	Immunofluorescence analysis of NIH/3T3 cells using ABCD1 antibody (CAB12141) at dilution of 1:100. Blue: DAPI for nuclear staining.