Product Name
BLOC1S3, siRNA
Full Product Name
BLOC1S3 siRNA (Human)
Product Synonym Names
BLOS3; Biogenesis of lysosome-related organelles complex 1 subunit 3; BLOC-1 subunit 3
Product Gene Name
BLOC1S3 sirna
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q6QNY0
Specificity
BLOC1S3 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
Purity/Purification
> 97%
Form/Format
Lyophilized powder
Quality Control
Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
Directions for Use
We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
Components
We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human BLOC1S3 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
Preparation and Storage
Shipped at 4 degree C. Store at -20 degree C for one year.
Negative Control
siRNA Negative Control (Catalog# MBS8241404) is a non-targeting 21 nt siRNA recommended as a negative control for experiments using targeted siRNA transfection.
Other Notes
Small volumes of BLOC1S3 sirna 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.
Related Product Information for
BLOC1S3 sirna
siRNA to inhibit BLOC1S3 expression using RNA interference
Applications Tested/Suitable for BLOC1S3 sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for BLOC1S3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_997715.1
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NCBI GenBank Nucleotide #
NM_212550.4
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UniProt Primary Accession #
Q6QNY0
[Other Products]
UniProt Secondary Accession #
B2RXB8[Other Products]
UniProt Related Accession #
Q6QNY0[Other Products]
Molecular Weight
21,256 Da
NCBI Official Full Name
biogenesis of lysosome-related organelles complex 1 subunit 3
NCBI Official Synonym Full Names
biogenesis of lysosomal organelles complex-1, subunit 3
NCBI Official Symbol
BLOC1S3 [Similar Products]
NCBI Official Synonym Symbols
RP; HPS8; BLOS3
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NCBI Protein Information
biogenesis of lysosome-related organelles complex 1 subunit 3
UniProt Protein Name
Biogenesis of lysosome-related organelles complex 1 subunit 3
Protein Family
Biogenesis of lysosome-related organelles complex
UniProt Gene Name
BLOC1S3 [Similar Products]
UniProt Synonym Gene Names
BLOS3; BLOC-1 subunit 3 [Similar Products]
UniProt Entry Name
BL1S3_HUMAN
NCBI Summary for BLOC1S3
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]
UniProt Comments for BLOC1S3
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; melanosome transport; positive regulation of natural killer cell activation; 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; neurite development
Disease: Hermansky-pudlak Syndrome 8
Research Articles on BLOC1S3
1. Observational study, meta-analysis, and genome-wide association study of gene-disease association and gene-gene interaction. (HuGE Navigator)
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
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