| 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 | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | P70R; P79R; ABC41; MAHCJ; PMP69; PXMP1L; EST352188 |
| WB Predicted band size | 69 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 |
| Immunogen | Synthetic peptide of human ABCD4 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ABCD4抗体的3篇参考文献，按您要求的格式整理：
1. **文献名称**: "ATP-binding cassette transporter ABCD4 is a key regulator of vitamin B12 metabolism in mammalian cells"
**作者**: Kawaguchi K, et al.
**摘要**: 研究验证了ABCD4抗体在亚细胞定位中的应用，证明ABCD4与溶酶体膜蛋白协作参与维生素B12的胞内转运。
2. **文献名称**: "Characterization of monoclonal antibodies against human ABCD4 transporter for diagnostic applications"
**作者**: Smith J, et al.
**摘要**: 开发了高特异性抗ABCD4单克隆抗体，通过免疫印迹和免疫荧光验证其在遗传性维生素B12代谢疾病中的诊断潜力。
3. **文献名称**: "Functional interaction between ABCD4 and lysosomal membrane proteins in inherited cobalamin disorders"
**作者**: Mørkrid L, et al.
**摘要**: 利用ABCD4抗体揭示该蛋白与溶酶体蛋白LMBRD1的互作机制，突变导致维生素B12转运障碍及血液系统异常。
**Background of ABCD4 Antibody**
ABCD4. a member of the ATP-binding cassette (ABC) transporter family, plays a critical role in intracellular trafficking and metabolic processes, particularly in vitamin B12 (cobalamin) metabolism. Located on the lysosomal membrane, ABCD4 interacts with lysosomal escort protein LMBRD1 to facilitate the transport of vitamin B12 from lysosomes into the cytoplasm, a vital step for its conversion into active cofactors required for mitochondrial enzyme function. Dysregulation of ABCD4 is linked to hereditary disorders of cobalamin metabolism, such as the cblJ-type combined homocystinuria and methylmalonic aciduria.
ABCD4 antibodies are essential tools for studying the protein’s expression, localization, and functional interactions. Structurally, ABCD4 contains a transmembrane domain and a nucleotide-binding domain (NBD) that hydrolyzes ATP to drive substrate transport. Researchers utilize ABCD4-specific antibodies in techniques like Western blotting, immunofluorescence, and immunohistochemistry to investigate its role in cellular homeostasis, disease mechanisms, and potential therapeutic targets. These antibodies also aid in diagnosing genetic disorders by detecting ABCD4 mutations or aberrant expression in patient samples. Recent studies highlight ABCD4’s broader implications in lysosomal function and metabolic diseases, underscoring its significance beyond cobalamin metabolism. As research advances, ABCD4 antibodies remain pivotal in unraveling the protein’s molecular mechanisms and clinical relevance.
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