| Product Name | HPS-6 Antibody (H-50) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 400-446 mapping within an internal region of HPS-6 of human origin |
| Modification Notes | Hermansky-Pudlak syndrome (HPS) is a rare, genetically heterogeneous, autosomal recessive disorder. It is characterized by oculocutaneous albinism, lysosomal storage defects and prolonged bleeding due to platelet storage pool deficiency. There are 10 HPS genes encoding HPS proteins that all interact within three distinct ubiquitously expressed protein complexes or biogenesis of lysosome-related organelle complexes. Defects in these genes cause HPS. Hermansky-Pudlak syndrome 6 protein (HPS-6), also designated Ruby-eye protein homolog (Ru) regulates the function and synthesis of lysosomes and other specialized organelles. HPS-6 interacts with HPS-5 to form the BLOC2 complex, also referred to as biogenesis of lysosome-related organelles complex-2. Defects in HPS6 can cause the Hermansky-Pudlak syndrome 6 (HPS6). |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of Hermansky-Pudlak Syndrome Protein 6 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 400-446 mapping within an internal region of HPS-6 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 400-446 mapping within an internal region of HPS-6 of human origin |
| Entrez Gene | 79803 |
|---|
| Application Notes | recommended for detection of Hermansky-Pudlak Syndrome Protein 6 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
