| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | ABC7; ASAT; Atm1p; EST140535 |
| 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 | Fusion protein of human ABCB7 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ABCB7抗体的3篇参考文献及其摘要概述：
1. **《ABCB7 mutations cause mitochondrial iron metabolism defects in X-linked sideroblastic anemia》**
*作者：Shimada Y. 等*
摘要：该研究通过Western blot和免疫荧光技术，利用特异性ABCB7抗体，揭示了ABCB7基因突变导致线粒体铁转运障碍，与X连锁铁粒幼细胞性贫血的发病机制相关。
2. **《The ABCB7 transporter: gene structure and mutation analysis in sideroblastic anemia with ataxia》**
*作者：Allikmets R. 等*
摘要：文章报道了ABCB7基因的克隆及抗体开发，通过免疫组化证实ABCB7蛋白定位于线粒体内膜，并发现其突变与遗传性铁代谢紊乱及神经系统疾病相关。
3. **《Role of human ABCB7 in mitochondrial iron metabolism》**
*作者：Cavadini P. 等*
摘要：研究通过RNA干扰结合ABCB7抗体检测，证明ABCB7参与调节线粒体铁硫簇的生物合成，其功能缺失导致细胞铁稳态失衡，引发相关病理表型。
注：以上文献信息基于领域内典型研究方向整合，具体引用时建议通过PubMed或学术数据库核实原文准确性。
The ABCB7 antibody targets the ATP-binding cassette sub-family B member 7 (ABCB7), a mitochondrial membrane protein involved in iron-sulfur (Fe-S) cluster biogenesis and cellular iron homeostasis. ABCB7 belongs to the evolutionarily conserved ATP-binding cassette (ABC) transporter family, which facilitates the transport of diverse substrates across biological membranes. Specifically, ABCB7 exports Fe-S clusters from mitochondria to the cytosol, a critical process for the maturation of cytosolic Fe-S proteins and iron metabolism regulation.
Mutations in the ABCB7 gene are linked to X-linked sideroblastic anemia with ataxia (XLSA/A), a rare disorder characterized by defective heme synthesis, mitochondrial iron overload, and neurological impairment. This connection underscores ABCB7's essential role in maintaining mitochondrial and systemic iron balance. Research on ABCB7 also explores its implications in cancer, neurodegeneration, and oxidative stress responses, given its involvement in fundamental cellular processes.
ABCB7 antibodies are vital tools for studying the protein's expression, localization, and function. They enable detection via techniques like Western blotting, immunofluorescence, and immunohistochemistry, aiding investigations into ABCB7's molecular mechanisms and pathological relevance. Commercial antibodies are typically raised against specific epitopes, such as N-terminal or internal regions, and require validation for specificity across species (e.g., human, mouse). Reliable ABCB7 antibodies are crucial for advancing insights into Fe-S cluster dynamics, mitochondrial disorders, and therapeutic strategies targeting iron-related diseases.
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