Overview
| Synonyms | ABC39,Abcd2,ABCD2,Adrenoleukodystrophy-like 1,Adrenoleukodystrophy-related protein,ALDL1,ALDR,ALDRP,ATP-binding cassette sub-family D member 2,hALDR |
| Swissprot | Q9UBJ2 |
| Source | Rabbit |
| Reactivity | Human,Mouse,Rat |
| Immunogen | Synthetic peptide of human ABCD2 |
| Application | IHC,ELISA |
| Recommended dilution | IHC 1:25-1:100 |
| Concentration | 0.2mg/mL |
| Clonality | Polyclonal |
Properties
| Cellular localization | |
| Tissue specificity | |
| Isotype | IgG |
| Purification | Affinity purification |
| Conjugation | Unconjugated |
| Storage instructions | Store at -20℃. Avoid freeze / thaw cycles. |
| Storage buffer | PBS with 0.05% sodium azide, 50% glycerol, PH7.3 |
| 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. The function of this peroxisomal membrane protein is unknown; however this protein is speculated to function as a dimerization partner of ABCD1 and/or other peroxisomal ABC transporters. Mutations in this gene have been observed in patients with adrenoleukodystrophy, a severe demyelinating disease. This gene has been identified as a candidate for a modifier gene, accounting for the extreme variation among adrenoleukodystrophy phenotypes. This gene is also a candidate for a complement group of Zellweger syndrome, a genetically heterogeneous disorder of peroxisomal biogenesis. |
