Biogenesis of lysosome-related organelles complex 1 subunit 3 (BLOC1S3), ELISA

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C.

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

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Small volumes of BLOC1S3 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS7218021 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Biogenesis of lysosome-related organelles complex 1 subunit 3 (BLOC1S3) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing BLOC1S3. The ELISA analytical biochemical technique of the MBS7218021 kit is based on BLOC1S3 antibody-BLOC1S3 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect BLOC1S3 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, BLOC1S3. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Signal Transduction

21,256 Da

biogenesis of lysosomal organelles complex-1, subunit 3

biogenesis of lysosome-related organelles complex 1 subunit 3; BLOC-1 subunit 3; reduced pigmentation, mouse, homolog of; biogenesis of lysosome-related organelles complex-1, subunit 3

Biogenesis of lysosome-related organelles complex 1 subunit 3

BLOS3; BLOC-1 subunit 3  [Similar Products]

BL1S3_HUMAN

This gene encodes a protein that is a component of the BLOC1 multi-subunit protein complex. This complex is necessary for the biogenesis of specialized organelles of the endosomal-lysosomal system, including platelet dense granules and melanosomes. Mutations in this gene cause Hermansky-Pudlak syndrome 8, a disease characterized by lysosomal storage defects, bleeding due to platelet storage pool deficiency, and oculocutaneous albinism. [provided by RefSeq, Jul 2008]

BLOC1S3: The BLOC-1 complex is required for normal biogenesis of lysosome-related organelles, such as platelet dense granules and melanosomes. Plays a role in intracellular vesicle trafficking. Defects in BLOC1S3 are the cause of Hermansky-Pudlak syndrome type 8 (HPS8). 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. Belongs to the BLOC1S3 family.

Protein type: Cell development/differentiation

Chromosomal Location of Human Ortholog: 19q13.32

Cellular Component: transport vesicle; cytosol

Molecular Function: protein binding

Biological Process: response to drug; platelet activation; secretion of lysosomal enzymes; eye development; pigmentation; platelet dense granule organization and biogenesis; melanosome organization and biogenesis; anterograde synaptic vesicle transport; post-Golgi vesicle-mediated transport; anterograde axon cargo transport; melanosome transport; neurite development; positive regulation of natural killer cell activation

Disease: Hermansky-pudlak Syndrome 8

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