| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ABCD1 |
| Uniprot No | P33897 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-745aa |
| 氨基酸序列 | MPVLSRPRPWRGNTLKRTAVLLALAAYGAHKVYPLVRQCLAPARGLQAPAGEPTQEASGVAAAKAGMNRVFLQRLLWLLRLLFPRVLCRETGLLALHSAALVSRTFLSVYVARLDGRLARCIVRKDPRAFGWQLLQWLLIALPATFVNSAIRYLEGQLALSFRSRLVAHAYRLYFSQQTYYRVSNMDGRLRNPDQSLTEDVVAFAASVAHLYSNLTKPLLDVAVTSYTLLRAARSRGAGTAWPSAIAGLVVFLTANVLRAFSPKFGELVAEEARRKGELRYMHSRVVANSEEIAFYGGHEVELALLQRSYQDLASQINLILLERLWYVMLEQFLMKYVWSASGLLMVAVPIITATGYSESDAEAVKKAALEKKEEELVSERTEAFTIARNLLTAAADAIERIMSSYKEVTELAGYTARVHEMFQVFEDVQRCHFKRPRELEDAQAGSGTIGRSGVRVEGPLKIRGQVVDVEQGIICENIPIVTPSGEVVVASLNIRVEEGMHLLITGPNGCGKSSLFRILGGLWPTYGGVLYKPPPQRMFYIPQRPYMSVGSLRDQVIYPDSVEDMQRKGYSEQDLEAILDVVHLHHILQREGGWEAMCDWKDVLSGGEKQRIGMARMFYHRPKYALLDECTSAVSIDVEGKIFQAAKDAGIALLSITHRPSLWKYHTHLLQFDGEGGWKFEKLDSAARLSLTEEKQRLEQQLAGIPKMQRRLQELCQILGEAVAPAHVPAPSPQGPGGLQGAST |
| 预测分子量 | 82,9 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300. |
| 稳定性 & 储存条件 | Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
| 复溶 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
以下是关于ABCD1重组蛋白的3篇参考文献概览：
1. **《Targeting of the peroxisomal ABC transporter ABCD1 requires the extension of its transmembrane domains》**
- **作者**：Kemp, S. 等
- **摘要**：研究ABCD1蛋白的跨膜结构域对其定位至过氧化物酶体的关键作用，通过重组蛋白实验揭示了结构完整性对功能的影响。
2. **《Functional characterization of ABCD1 mutations in X-linked adrenoleukodystrophy》**
- **作者**：Morita, M. 等
- **摘要**：通过重组ABCD1蛋白表达，分析不同突变对蛋白转运极长链脂肪酸(VLCFA)能力的影响，为肾上腺脑白质营养不良的分子机制提供依据。
3. **《Adenovirus-mediated gene transfer of ABCD1 cDNA in human fibroblasts from X-ALD patients》**
- **作者**：Cartier-Lacave, S. 等
- **摘要**：利用腺病毒载体在患者成纤维细胞中表达重组ABCD1蛋白，验证其对VLCFA代谢的修复效果，探索潜在基因治疗策略。
如需更多文献，可进一步检索PubMed或专业数据库。
**Background of ABCD1 Recombinant Protein**
ABCD1 (ATP-binding cassette subfamily D member 1) is a peroxisomal membrane protein encoded by the *ABCD1* gene, belonging to the ATP-binding cassette (ABC) transporter superfamily. This protein plays a critical role in transporting very long-chain fatty acids (VLCFAs) into peroxisomes for β-oxidation, a process essential for lipid metabolism and cellular energy homeostasis. Mutations in *ABCD1* lead to X-linked adrenoleukodystrophy (X-ALD), a rare neurodegenerative disorder characterized by the accumulation of VLCFAs in tissues, adrenal insufficiency, and progressive demyelination in the central nervous system.
Recombinant ABCD1 protein is engineered using molecular cloning techniques, typically expressed in heterologous systems like *E. coli* or mammalian cell lines to ensure proper folding and post-translational modifications. Its production enables detailed studies on ABCD1’s structure-function relationships, substrate specificity, and interaction partners. Researchers utilize this recombinant protein to investigate molecular mechanisms underlying X-ALD pathogenesis, including impaired peroxisomal transport and metabolic dysregulation.
Additionally, ABCD1 recombinant protein serves as a tool for drug screening and therapeutic development, such as validating small molecules that enhance residual transporter activity or restore protein stability in mutant cells. It also aids in generating antibodies for diagnostic assays, improving early detection of X-ALD. Recent studies explore gene therapy approaches, where recombinant ABCD1 variants are tested for functional rescue in preclinical models.
Overall, ABCD1 recombinant protein is vital for advancing our understanding of peroxisomal biology and developing targeted therapies for X-ALD, bridging gaps between genetic defects and clinical interventions.
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