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Highest antigen sequence indentity to the following orthologs: Mouse (75%), Rat (75%).
This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-63938. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
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. HSP-1 is a component of multiple cytoplasmic organelles and is important for their normal development and function. It plays a role in intracellular protein sorting.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: BLOC-3 complex member HPS1; Hermansky-Pudlak syndrome 1 protein; MGC5277
基因别名: BLOC3S1; HPS; HPS1
UniProt ID: (Human) Q92902
Entrez Gene ID: (Human) 3257