DTNBP1 anticorps (N-Term)
Aperçu rapide pour DTNBP1 anticorps (N-Term) (ABIN966013)
Antigène
Voir toutes DTNBP1 AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- N-Term
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Purification
- Purified by antigen-specific affinity chromatography.
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Immunogène
- Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to N-terminal residues of human DTNBP1(Dystrobrevin binding protein 1)
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Indications d'application
- ELISA, Western blotting: 1µg/ml for 2hrs.
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Restrictions
- For Research Use only
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Format
- Liquid
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Buffer
- This antibody is stored in PBS, 50% glycerol
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Agent conservateur
- Sodium azide
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Précaution d'utilisation
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Stock
- -20 °C
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- DTNBP1 (Dystrobrevin Binding Protein 1 (DTNBP1))
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Autre désignation
- DTNBP1(Dystrobrevin binding protein 1)
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Sujet
- DTNBP1(Dystrobrevin binding protein 1)plays a role in the biogenesis of lysosome-related organelles such as platelet dense granule and melanosomes. Dystrobrevin binding protein 1 binds to alpha and beta dystrobrevins that are components of the dystrophin-associated protein complex (DPC). It interacts with pallidin and MUTED. Dystrobrevin binding protein 1 is a part of the biogenesis of lysosome-related organelles complex 1 (BLOC-1). Defects in DTNBP1 are the cause of Hermansky-Pudlak syndrome 7 (HPS7). 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. Dystrobrevin binding protein 1 belongs to the dysbindin family.
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Pathways
- Synaptic Membrane, Regulation of G-Protein Coupled Receptor Protein Signaling
Antigène
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