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Antibody Product Overview
| ELK.No | ES15672 |
| Product name | HPS6 rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 79803 |
| Human Swiss-Prot | Q86YV9 |
| Source | Rabbit |
| Isotype | IgG |
| Target | HPS6 |
| Fields | |
| Gene name | HPS6 |
| Protein name | HPS6 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 20170 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q8BLY7 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 309446 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q7M733 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human HPS6 AA range: 168-218 |
| Specificity | This antibody detects endogenous levels of HPS6 at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 85kD |
| Observed band (KD) | |
| Background | 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 | disease:Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6) [MIM:203300]. 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.,function:May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules.,subunit:Directly interacts with HSP5 in a complex known as biogenesis of lysosome-related organelles complex-2 (or BLOC2)., |
| Subcellular location | Microsome membrane . Cytoplasm, cytosol . Early endosome membrane . Lysosome membrane . |
| Expression | Ubiquitous. |
Additional Images
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Western blot analysis of lysates from HCT116 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES15672-50
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