DTNBP1 anticorps (N-Term)

N° du produit ABIN966013

Cet anticorps Lapin Polyclonal détecte spécifiquement DTNBP1 dans IHC. Il présente une réactivité envers Humain et Souris.

Aperçu rapide pour DTNBP1 anticorps (N-Term) (ABIN966013)

Antigène: DTNBP1 (Dystrobrevin Binding Protein 1 (DTNBP1))

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp DTNBP1 est non-conjugé

Application: Immunohistochemistry (IHC)

Détail du produit anti-DTNBP1 anticorps

Épitope: N-Term

Purification: Purified by antigen-specific affinity chromatography.

Immunogène: Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to N-terminal residues of human DTNBP1(Dystrobrevin binding protein 1)

Information d'application

Indications d'application: ELISA, Western blotting: 1µg/ml for 2hrs.

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: This antibody is stored in PBS, 50% glycerol

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: -20 °C

Détails sur DTNBP1

Antigène: DTNBP1 (Dystrobrevin Binding Protein 1 (DTNBP1))

Autre désignation: DTNBP1(Dystrobrevin binding protein 1)

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.

Pathways: Synaptic Membrane, Regulation of G-Protein Coupled Receptor Protein Signaling