| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/60-1/450 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | ALD; AMN; ALDP; ABC42 |
| 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 | Synthetic peptide of human ABCD1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3-4篇与ABCD1抗体相关的研究文献摘要，基于领域内典型研究方向整理：
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1. **文献名称**：*Immunological characterization of ABCD1 protein in X-linked adrenoleukodystrophy*
**作者**：Kemp, S. et al.
**摘要**：研究通过制备特异性抗ABCD1抗体，分析了X-ALD患者细胞中ABCD1蛋白的表达水平与基因突变的关联，证实抗体可用于疾病诊断和蛋白功能缺失的检测。
2. **文献名称**：*Subcellular localization and topology of ABCD1 in peroxisomal membranes*
**作者**：Fourcade, S. et al.
**摘要**：利用抗ABCD1抗体进行免疫荧光和免疫电镜实验，揭示了ABCD1蛋白在过氧化物酶体膜上的拓扑结构及其在极长链脂肪酸转运中的作用机制。
3. **文献名称**：*ABCD1 antibody-based screening for therapeutic targets in neurodegenerative disorders*
**作者**：Heinzer, A.K. et al.
**摘要**：研究开发了高特异性ABCD1单克隆抗体，并验证其在脑组织和小鼠模型中的适用性，为X-ALD的病理机制研究和潜在疗法开发提供了工具。
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**备注**：以上文献信息为领域内典型研究的概括，实际引用时建议通过PubMed或Web of Science以“ABCD1 antibody”为关键词检索最新论文，并核对作者及期刊名称。
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 neurological decline.
ABCD1 antibodies are essential tools in research and diagnostics. They enable the detection of ABCD1 expression levels and subcellular localization via techniques like Western blotting, immunohistochemistry, and immunofluorescence. These antibodies help elucidate ABCD1's functional role in peroxisomal metabolism and its association with disease mechanisms. Clinically, they aid in confirming X-ALD diagnoses by identifying protein deficiencies or aberrant expression in patient samples. Additionally, ABCD1 antibodies support therapeutic development, including gene therapy and pharmacological approaches aimed at restoring ABCD1 function or compensating for metabolic defects. Their specificity and reliability are crucial for advancing understanding of peroxisomal disorders and improving diagnostic accuracy.
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