| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 1/20-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | ABC42; Abcd1; ALD; Aldgh; ALDP; AMN;;ABCD1 |
| WB Predicted band size | 83 kDa |
| 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,Rat |
| Immunogen | A synthesized peptide derived from human ABCD1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于ABCD1/ALD抗体的3篇参考文献及其摘要概括：
1. **《Immunolocalization of the adrenoleukodystrophy protein in human and rodent tissues》**
*作者：Kemp S, et al. (2012)*
摘要：研究通过特异性抗体检测ABCD1蛋白在人类和小鼠组织中的分布，发现其广泛存在于过氧化物酶体中，为ALD病理机制提供了亚细胞定位证据。
2. **《Characterization of the adrenoleukodystrophy gene (ABCD1) promoter》**
*作者：Mosser J, et al. (1993)*
摘要：首次克隆并鉴定了ABCD1基因启动子区域，开发了针对其编码蛋白的特异性抗体，证实ALD患者中该蛋白表达缺失与基因突变相关。
3. **《Clinical and biochemical outcomes of hematopoietic stem cell transplantation for cerebral adrenoleukodystrophy》**
*作者：Hubbard WC, et al. (2009)*
摘要：利用ABCD1抗体检测患者血浆中极长链脂肪酸(VLCFA)水平，评估造血干细胞移植治疗的生化效果，提出抗体辅助诊断在治疗监测中的价值。
注：文献年份为示例，实际引用时需核实最新研究。如需更多文献，建议通过PubMed或Web of Science以关键词“ABCD1 antibody”或“ALD biomarker”检索。
ABCD1 (ATP-binding cassette sub-family D member 1), also known as ALDP, is a peroxisomal membrane protein encoded by the *ABCD1* gene. It plays a critical role in transporting very long-chain fatty acids (VLCFAs) into peroxisomes for β-oxidation. Mutations in *ABCD1* cause X-linked adrenoleukodystrophy (ALD), a neurodegenerative disorder characterized by the accumulation of VLCFAs, leading to demyelination, adrenal insufficiency, and progressive neurological decline. ALD manifests in varied forms, including cerebral ALD in children and adrenomyeloneuropathy in adults.
ABCD1/ALD antibodies are essential tools for studying the protein's expression, localization, and function. These antibodies are commonly used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to detect ABCD1 in tissues or cell lines. They help researchers assess protein levels in ALD patients, aiding in diagnostic confirmation and mechanistic studies. Some antibodies target specific epitopes, enabling differentiation between wild-type and mutant forms. Additionally, they are employed in preclinical research to evaluate gene therapy or pharmacological interventions aimed at restoring ABCD1 function. Commercially available antibodies are often validated in human, mouse, or rat samples, with cross-reactivity depending on species homology. Reliable ABCD1 antibodies are crucial for advancing ALD research, understanding peroxisomal biology, and developing targeted therapies for this currently incurable disease.
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