| 產(chǎn)品編號 | bs-17384R |
| 英文名稱 | HPS6 Rabbit pAb |
| 中文名稱 | Hermansky-Pudlak綜合征蛋白6抗體 |
| 別 名 | FLJ22501; Hermansky Pudlak syndrome 6 protein; Hermansky-Pudlak syndrome 6 protein; HPS6; HPS6_HUMAN; MGC20522; Ru; Ruby eye protein homolog; Ruby-eye protein homolog. |
| 研究領(lǐng)域 | 免疫學(xué) 信號轉(zhuǎn)導(dǎo) 結(jié)合蛋白 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human (predicted: Mouse,Rat,Pig,Cow,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 83 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HPS6: 501-600/775 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
This intronless gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. This protein interacts with Hermansky-Pudlak syndrome 5 protein. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 6. [provided by RefSeq, Jul 2008] Function: May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Subcellular Location: Microsome membrane. Cytoplasm > cytosol. Early endosome membrane. Tissue Specificity: Ubiquitous. DISEASE: Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6) [MIM:614075]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. SWISS: Q86YV9 Gene ID: 79803 Database links: Entrez Gene: 79803 Human Omim: 607522 Human SwissProt: Q86YV9 Human Unigene: 125133 Human |
| 產(chǎn)品圖片 |
Sample:
293T(Human) Cell Lysate at 30 ug
Primary: Anti-HPS6 (bs-17384R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 83 kD
Observed band size: 83 kD
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