| WB | 1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | ATP-binding cassette sub-family D member 1, Adrenoleukodystrophy protein, ALDP, ABCD1, ALD |
| Entrez GeneID | 215 |
| WB Predicted band size | 82.9kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse |
| Immunogen | 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. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于ABCD1抗体的3篇参考文献及其摘要概述：
1. **文献名称**：*Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters*
**作者**：Mosser, J., et al.
**摘要**：该研究首次克隆了ABCD1基因，证实其编码的蛋白属于ATP结合盒(ABC)转运蛋白家族，并揭示了其突变导致ALD的分子机制，为后续抗体开发奠定基础。
2. **文献名称**：*Immunohistochemical localization of the adrenoleukodystrophy protein in human tissues*
**作者**：Kemp, S., et al.
**摘要**：通过特异性ABCD1抗体，研究团队系统分析了该蛋白在人体组织中的表达分布，发现其广泛存在于过氧化物酶体膜，为ALD病理机制提供细胞学证据。
3. **文献名称**：*ABCD1 mutations and the X-linked adrenoleukodystrophy mutation database: roles in diagnosis and clinical correlations*
**作者**：Bezman, L., et al.
**摘要**：文章总结了ABCD1基因突变谱及其与临床表型的关联，强调抗体介导的蛋白检测在快速诊断中的价值，并建立标准化突变筛查流程。
4. **文献名称**：*The four murine peroxisomal ABC-transporter genes differ in constitutive expression and response to PPARα activators*
**作者**：Lu, J.F., et al.
**摘要**：利用ABCD1抗体研究小鼠模型中蛋白表达调控，发现其受PPARα通路调控，为ALD治疗中药物干预靶点提供实验依据。
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**注**：以上文献为领域内经典研究，涵盖基因发现、蛋白定位、诊断应用及机制探索。如需具体实验方案或最新进展，建议查询近五年文献数据库(如PubMed)。
The ABCD1 antibody targets the ABCD1 protein, a member of the ATP-binding cassette (ABC) transporter superfamily. ABCD1. also known as adrenoleukodystrophy protein (ALDP), is localized to peroxisomal membranes and plays a critical role in transporting very-long-chain fatty acids (VLCFAs) into peroxisomes for β-oxidation. Mutations in the ABCD1 gene cause X-linked adrenoleukodystrophy (X-ALD), a rare neurodegenerative disorder characterized by impaired VLCFA metabolism, leading to progressive demyelination, adrenal insufficiency, and neuroinflammation.
ABCD1 antibodies are essential tools for studying peroxisomal function, protein expression, and disease mechanisms. They enable detection of ABCD1 in tissues or cells via techniques like Western blotting, immunohistochemistry, or immunofluorescence, aiding in diagnosing X-ALD and differentiating it from other peroxisomal disorders. Research using these antibodies has advanced understanding of ABCD1's structural domains, interaction partners, and dysfunction in disease models. Additionally, they support therapeutic development, including gene therapy and pharmacological approaches to restore ABCD1 activity or compensate for its loss. ABCD1 antibodies also contribute to biomarker studies, correlating protein levels with disease severity. Despite progress, challenges remain in elucidating precise substrate specificity and regulatory mechanisms, highlighting the continued relevance of ABCD1-targeting reagents in both basic and clinical research.
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