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DTNBP1 pAb

DTNBP1 pAb

Cat no: BS6806

Supplier: Bioworld Technology, Inc.
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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. HPS is a result of defects in various cytoplasmic organelles such as melanosomes, platelet dense granules and lysosomes. The HPS proteins, including HPS-1-6 and Dysbindin (also designated HPS-7), all interact within three distinct, ubiquitously expressed protein complexes or biogenesis of lysosome-related organelle complexes. Defects in the genes encoding for these proteins are the cause of HPS. Dysbindin binds to dystrobrevins in the dystrophin-associated protein complex (DPC) complex. Dysbindin is a cytoplasmic protein. Isoforms 1 and 2 are the result of alternative splicing.
Catalogue number: BS6806
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Immunohistochemistry, Western Blot
Size: 100ug/100ul
Swiss prot: Q96EV8
Dilutions: WB: 1:500~1:2000 IHC: 1:50~1:200
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Recombinant full length Human DTNBP1.
Species: DTNBP1 pAb detects endogenous levels of DTNBP1 protein.
Molecular weight: ~ 39 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: DTBP1; DTNBP1; Dysbindin; Dysbindin-1; Dystrobrevin binding protein 1; Dystrobrevin-binding protein 1; Hermansky Pudlak syndrome 7 protein; Hermansky-Pudlak syndrome 7 protein; HPS7; HPS7 protein;
Additional info: For research use only, not for use in diagnostic procedure.

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