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ABCD1 Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| FC, IHC-P, WB, E |
|---|---|
| Primary Accession | P33897 |
| Other Accession | P48410, NP_000024.2, D3ZHR2 |
| Reactivity | Human |
| Predicted | Mouse, Rat |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Calculated MW | 82937 Da |
| Antigen Region | 257-285 aa |
| Gene ID | 215 |
|---|---|
| Other Names | ATP-binding cassette sub-family D member 1, Adrenoleukodystrophy protein, ALDP, ABCD1, ALD |
| Target/Specificity | This ABCD1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 257-285 amino acids from the Central region of human ABCD1. |
| Dilution | WB~~1:2000 IHC-P~~1:50~100 FC~~1:10~50 |
| Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
| Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | ABCD1 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | ABCD1 (HGNC:61) |
|---|---|
| Synonyms | ALD |
| Function | ATP-dependent transporter of the ATP-binding cassette (ABC) family involved in the transport of very long chain fatty acid (VLCFA)- CoA from the cytosol to the peroxisome lumen (PubMed:11248239, PubMed:15682271, PubMed:16946495, PubMed:18757502, PubMed:21145416, PubMed:23671276, PubMed:29397936, PubMed:33500543). Coupled to the ATP- dependent transporter activity has also a fatty acyl-CoA thioesterase activity (ACOT) and hydrolyzes VLCFA-CoA into VLCFA prior their ATP- dependent transport into peroxisomes, the ACOT activity is essential during this transport process (PubMed:29397936, PubMed:33500543). Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation (PubMed:21145416, PubMed:23671276). Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance. Controls also the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization. And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (By similarity). |
| Cellular Location | Peroxisome membrane; Multi-pass membrane protein. Mitochondrion membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein Endoplasmic reticulum membrane; Multi- pass membrane protein |
Thousands of laboratories across the world have published research that depended on the performance of antibodies from Abcepta to advance their research. Check out links to articles that cite our products in major peer-reviewed journals, organized by research category.
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Provided below are standard protocols that you may find useful for product applications.
Background
ABCD1 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.
References
Matsukawa, T., et al. Neurogenetics (2010) In press :
Xie, H.H., et al. Zhonghua Yi Xue Yi Chuan Xue Za Zhi 27(2):144-148(2010)
Li, J.Y., et al. J. Neurol. Sci. 290 (1-2), 163-165 (2010) :
Hour, T.C., et al. Int. J. Biol. Markers 24(3):171-178(2009)
Shukla, P., et al. J. Child Neurol. 24(7):857-860(2009)
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