DTNBP1 Antikörper (N-Term)

Produktnummer ABIN966013

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch DTNBP1 in IHC. Er zeigt eine Reaktivität gegenüber Human und Maus.

Kurzübersicht für DTNBP1 Antikörper (N-Term) (ABIN966013)

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

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser DTNBP1 Antikörper ist unkonjugiert

Applikation: Immunohistochemistry (IHC)

Produktdetails anti-DTNBP1 Antikörper

Bindungsspezifität: N-Term

Aufreinigung: Purified by antigen-specific affinity chromatography.

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

Anwendungsinformationen

Applikationshinweise: ELISA, Western blotting: 1µg/ml for 2hrs.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

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

Konservierungsmittel: Sodium azide

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

Lagerung: -20 °C

Details zu DTNBP1

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

Andere Bezeichnung: DTNBP1(Dystrobrevin binding protein 1)

Hintergrund: 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