Peptide ELISA: > 1/32,000. Western Blot: 1-3 μg/mL. Detects a band of Approx 110 kDa in A431 cell lysate(Predicted Molecular Weight: 114 kDa). Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
限制
仅限研究用
浓度
0.5 mg/mL
缓冲液
Tris saline, pH 7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer.
储存液
Sodium azide
注意事项
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
HPS3 is involved in early stages of melanosome biogenesis and maturation. Defects in HPS3 are the cause of the cocoa (coa) mutant, and of Hermansky-Pudlak syndrome type 3 (HPS3). HPS3 is an 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.Synonyms: Hermansky-Pudlak syndrome 3