| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/20-1/100 | 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 | F5F8D; SDNSF; F5F8D2; LMAN1IP |
| 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 MCFD2 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3条关于MCFD2抗体的模拟参考文献(内容为虚构示例，实际文献需通过学术数据库查询)：
1. **文献名称**: "MCFD2 interacts with ERGIC-53 to regulate coagulation factor V and VIII trafficking"
**作者**: Zhang L, et al.
**摘要**: 研究利用MCFD2特异性抗体揭示了MCFD2与内质网-高尔基体中间区蛋白ERGIC-53的相互作用机制，证明二者共同参与凝血因子V和VIII的细胞内运输，突变导致联合凝血因子缺乏症。
2. **文献名称**: "Functional characterization of MCFD2 mutations in congenital coagulation disorders"
**作者**: Tanaka K, et al.
**摘要**: 通过MCFD2抗体免疫印迹和免疫荧光技术，发现患者中MCFD2基因突变导致蛋白质稳定性下降，影响其与ERGIC-53的结合能力，进而引发凝血功能障碍。
3. **文献名称**: "Development of a monoclonal antibody for MCFD2: Diagnostic application in F5F8D cases"
**作者**: Wang Y, et al.
**摘要**: 报道一种高特异性抗人MCFD2单克隆抗体的开发，该抗体可用于ELISA和免疫组化检测，显著提高遗传性凝血因子V/VIII联合缺乏症(F5F8D)的临床诊断准确性。
(注：以上为模拟内容，真实文献请通过PubMed、Google Scholar等平台检索。)
The MCFD2 (Multiple Coagulation Factor Deficiency 2) antibody is a tool used to detect the MCFD2 protein, which plays a critical role in intracellular transport of coagulation factors V and VIII. MCFD2. encoded by the MCFD2 gene, forms a complex with LMAN1 (ERGIC-53) to facilitate the endoplasmic reticulum-to-Golgi transport of these clotting factors. Mutations in MCFD2 or LMAN1 are linked to combined factor V and VIII deficiency (F5F8D), a rare autosomal recessive bleeding disorder characterized by reduced levels of both factors.
MCFD2 antibodies are essential for studying the protein's expression, localization, and interaction partners. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate MCFD2's role in coagulation pathways and cellular trafficking mechanisms. Research utilizing these antibodies has advanced understanding of how MCFD2-LMAN1 complexes ensure proper secretion of factors V and VIII, and how their dysfunction leads to bleeding phenotypes.
Clinically, MCFD2 antibodies may aid in diagnosing F5F8D by confirming protein deficiencies or mutations. Their application extends to exploring therapeutic strategies, including gene therapy or protein replacement, to address the underlying molecular defects. As a research reagent, MCFD2 antibodies remain vital for dissecting coagulation biology and related disorders.
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