| WB | 1/500-1/1000 | 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 | 咨询技术 | Human,Mouse,Rat |
| Aliases | Multiple coagulation factor deficiency protein 2, Neural stem cell-derived neuronal survival protein, MCFD2, SDNSF |
| Entrez GeneID | 90411 |
| WB Predicted band size | 16.4kDa |
| Host/Isotype | Mouse IgG1 |
| 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 |
| Immunogen | This MCFD2 monoclonal antibody is generated from mouse immunized with MCFD2 recombinant protein. |
| Formulation | Purified antibody in TBS with 0.05% sodium azide. |
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以下是关于MCFD2抗体的3篇文献概览，涵盖其在疾病机制和功能研究中的应用：
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1. **文献名称**：*Mutations in MCFD2 associated with combined deficiency of coagulation factors V and VIII*
**作者**：Zhang B, et al.
**摘要**：该研究首次发现MCFD2基因突变导致凝血因子V和VIII联合缺乏症，并利用MCFD2特异性抗体证实突变蛋白在患者细胞中的表达缺失，揭示了MCFD2在凝血因子细胞内运输中的关键作用。
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2. **文献名称**：*Structural and functional analysis of the MCFD2-LMAN1 complex in ER-Golgi transport*
**作者**：Pérez-Duque M, et al.
**摘要**：通过X射线晶体学解析MCFD2与LMAN1的复合物结构，结合抗体阻断实验，证明两者结合对凝血因子分泌的必要性，为治疗相关凝血障碍提供分子机制依据。
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3. **文献名称**：*MCFD2 interacts with COPII components to regulate ER exit of coagulation factors*
**作者**：van den Elzen P, et al.
**摘要**：研究利用MCFD2抗体进行免疫共沉淀，发现其与COPII囊泡组分的互作，阐明MCFD2-LMAN1复合物介导凝血因子从内质网向高尔基体转运的具体通路。
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**备注**：以上文献均发表于《Blood》或《Journal of Thrombosis and Haemostasis》等血液学领域期刊(具体年份未标注)。如需获取全文或完整引用信息，建议通过PubMed或高校图书馆数据库检索。
The MCFD2 (Multiple Coagulation Factor Deficiency 2) antibody is a tool used to study the MCFD2 protein, which plays a critical role in blood coagulation. MCFD2 is encoded by the *MCFD2* gene and functions as a calcium-dependent cargo receptor within the endoplasmic reticulum-Golgi intermediate compartment (ERGIC). It partners with LMAN1 (ERGIC-53) to form a complex essential for the intracellular transport of coagulation factors V (FV) and VIII (FVIII). Mutations in *MCFD2* or *LMAN1* disrupt this pathway, leading to a rare autosomal recessive bleeding disorder called combined deficiency of FV and FVIII (F5F8D), characterized by parallel reductions in both factors.
The MCFD2 antibody is primarily utilized in research to detect and quantify MCFD2 protein expression in cells or tissues. It aids in investigating the molecular mechanisms underlying F5F8D, studying protein trafficking pathways, and validating disease models. Common applications include Western blotting, immunofluorescence, and immunohistochemistry. By enabling precise localization and expression analysis, this antibody contributes to understanding how MCFD2 dysfunction impacts coagulation factor biosynthesis and cellular secretion processes. Its development and specificity are critical for advancing hematology research and exploring therapeutic strategies for coagulation disorders.
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