| WB | 1/1000-1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/500 | 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 | Cytoplasmic dynein 1 heavy chain 1, Cytoplasmic dynein heavy chain 1, Dynein heavy chain, cytosolic, DYNC1H1, DHC1, DNCH1, DNCL, DNECL, DYHC, KIAA0325 |
| Entrez GeneID | 1778 |
| WB Predicted band size | 532.4kDa |
| 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 | This DYNC1H1 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 4202-4236 amino acids from the C-terminal region of human DYNC1H1. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于DYNC1H1抗体的3篇文献摘要简述：
1. **文献名称**：*Mutations in DYNC1H1 cause severe intellectual disability and neuronal migration defects*
**作者**：Willemsen MH, et al.
**摘要**：该研究通过全外显子测序发现DYNC1H1基因突变与严重智力障碍和皮质发育畸形相关，利用DYNC1H1抗体进行免疫荧光和Western blot实验，证实突变导致蛋白质表达异常及神经元迁移障碍。
2. **文献名称**：*DYNC1H1 mutations associated with dominant spinal muscular atrophy alter microtubule binding and axonal transport*
**作者**：Hafezparast M, et al.
**摘要**：研究揭示了DYNC1H1突变通过破坏其与微管的相互作用导致轴突运输缺陷，进而引发脊髓性肌萎缩。通过DYNC1H1特异性抗体进行免疫沉淀和细胞定位分析，验证突变对动力蛋白复合体功能的影响。
3. **文献名称**：*Impaired cargo trafficking in DYNC1H1-linked neurodevelopmental disorders*
**作者**：Hoang HT, et al.
**摘要**：该文献利用患者来源的细胞系和DYNC1H1抗体进行功能研究，发现突变导致神经元内线粒体和囊泡运输受阻，强调了DYNC1H1在维持神经细胞胞内运输及突触功能中的关键作用。
The DYNC1H1 antibody is a crucial tool in studying the cytoplasmic dynein 1 heavy chain 1. a protein encoded by the *DYNC1H1* gene. This protein serves as a core component of the cytoplasmic dynein complex, a motor protein responsible for retrograde intracellular transport along microtubules. It plays vital roles in mitotic spindle organization, organelle positioning, and neuronal axon trafficking. Mutations in *DYNC1H1* are linked to neurodevelopmental disorders (e.g., spinal muscular atrophy, intellectual disability) and neuromuscular diseases (e.g., Charcot-Marie-Tooth disease), making its study essential for understanding disease mechanisms.
DYNC1H1 antibodies are widely used in techniques like Western blotting, immunofluorescence, and immunohistochemistry to detect protein expression, localization, and interaction partners. Researchers employ these antibodies to investigate dynein dysfunction in cellular models, animal studies, or patient-derived samples. Specificity and validation (e.g., knockout controls) are critical, as DYNC1H1 shares structural homology with other dynein isoforms. Commercial antibodies often target conserved regions, such as the N-terminal domain or ATP-binding sites.
Recent studies also explore DYNC1H1's role in cancer metastasis and viral pathogen transport, expanding its biomedical relevance. By enabling precise protein analysis, DYNC1H1 antibodies contribute to advancing both basic cell biology and clinical research.
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