| 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 | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | SUTAL; BLOC2S1 |
| WB Predicted band size | 114 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 |
| Immunogen | Fusion protein of human HPS3 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于HPS3抗体的模拟参考文献示例(非真实文献，仅供格式参考)：
1. **"HPS3基因编码的蛋白功能及其抗体在溶酶体研究中的应用"**
*作者：Smith A, et al.*
摘要：本研究通过构建HPS3基因敲除小鼠模型，利用HPS3特异性抗体检测其在细胞内的定位，发现HPS3蛋白参与调控溶酶体相关细胞器的形成，抗体可用于诊断Hermansky-Pudlak综合征3型。
2. **"HPS3抗体在皮肤黑色素细胞中的表达分析"**
*作者：Zhang L, et al.*
摘要：通过免疫组化和Western blot技术，验证HPS3抗体在黑色素细胞中的特异性，发现HPS3蛋白缺失与黑色素颗粒运输异常相关，为色素沉着疾病的机制研究提供工具。
3. **"新型HPS3单克隆抗体的开发及其临床诊断潜力"**
*作者：Tanaka K, et al.*
摘要：报道一种高特异性HPS3单克隆抗体的制备方法，通过患者血液样本验证其诊断敏感性，证明该抗体可用于快速筛查Hermansky-Pudlak综合征3型患者。
4. **"HPS3与HPS6蛋白相互作用的抗体介导研究"**
*作者：Johnson R, et al.*
摘要：使用HPS3和HPS6双抗体共沉淀技术，揭示两种蛋白在细胞内形成复合物，调控囊泡运输过程，为溶酶体疾病治疗提供新靶点。
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注：以上内容为模拟示例，实际文献需通过学术数据库(如PubMed、Web of Science)检索。
**Background of HPS3 Antibody**
The HPS3 antibody targets the Hermansky-Pudlak syndrome type 3 (HPS3) protein, which is associated with a rare autosomal recessive disorder called Hermansky-Pudlak syndrome (HPS). HPS encompasses a group of genetic conditions characterized by defects in lysosome-related organelles, leading to symptoms such as oculocutaneous albinism, bleeding diathesis, and, in some subtypes, pulmonary fibrosis or immunodeficiency. HPS3. specifically, is caused by mutations in the *HPS3* gene located on chromosome 3q24. This gene encodes a protein involved in intracellular trafficking and biogenesis of melanosomes, platelet dense granules, and other lysosome-related organelles.
HPS3 antibodies are primarily used in research to study the expression, localization, and functional role of the HPS3 protein in cellular processes. They are employed in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate molecular mechanisms underlying HPS pathophysiology. Commercially available HPS3 antibodies are typically developed in animal hosts (e.g., rabbits or mice) using immunogenic peptide sequences derived from the human HPS3 protein.
These antibodies aid in diagnosing HPS subtypes and understanding genotype-phenotype correlations. Studies using HPS3 antibodies have also contributed to elucidating interactions between HPS3 and other HPS-associated proteins (e.g., HPS1. HPS4) in protein complexes like BLOC-2 (Biogenesis of Lysosome-related Organelles Complex-2), which regulates organelle maturation and trafficking. Overall, HPS3 antibodies serve as critical tools for both basic research and clinical investigations into HPS and related lysosomal disorders.
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