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COL6A3, siRNA

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產(chǎn)品名稱: COL6A3, siRNA
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COL6A3, siRNA


COL6A3, siRNA  的詳細(xì)介紹
Product Name

COL6A3, siRNA

Full Product Name

COL6A3 siRNA (Human)

Product Synonym Names
Collagen alpha-3(VI) chain
Product Gene Name

COL6A3 sirna

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
120250
3D Structure
ModBase 3D Structure for P12111
Host
Synthetic
Species Reactivity
Human
Specificity
COL6A3 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 COL6A3 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 COL6A3 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.
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Related Product Information for
COL6A3 sirna
siRNA to inhibit COL6A3 expression using RNA interference
Applications Tested/Suitable for COL6A3 sirna
RNA Interference (RNAi)
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NCBI/Uniprot data below describe general gene information for COL6A3. It may not necessarily be applicable to this product.
NCBI GI #
55743098
NCBI GeneID
1293
NCBI Accession #
NP_004360.2 [Other Products]
NCBI GenBank Nucleotide #
NM_004369.3 [Other Products]
UniProt Primary Accession #
P12111 [Other Products]
UniProt Secondary Accession #
Q16501; Q53QF4; Q53QF6; A8MT30; B4E3U5; B7ZMJ7; E9PFQ6; E9PGQ9[Other Products]
UniProt Related Accession #
P12111[Other Products]
Molecular Weight
134,707 Da
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NCBI Official Full Name
collagen alpha-3(VI) chain isoform 1
NCBI Official Synonym Full Names
collagen, type VI, alpha 3
NCBI Official Symbol
COL6A3??[Similar Products]
NCBI Official Synonym Symbols
DYT27
??[Similar Products]
NCBI Protein Information
collagen alpha-3(VI) chain
UniProt Protein Name
Collagen alpha-3(VI) chain
Protein Family
Collagen
UniProt Gene Name
COL6A3??[Similar Products]
UniProt Entry Name
CO6A3_HUMAN
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NCBI Summary for COL6A3
This gene encodes the alpha-3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is much larger than the alpha-1 and -2 chains. This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all the alpha chains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in the type VI collagen genes are associated with Bethlem myopathy, a rare autosomal dominant proximal myopathy with early childhood onset. Mutations in this gene are also a cause of Ullrich congenital muscular dystrophy, also referred to as Ullrich scleroatonic muscular dystrophy, an autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Multiple transcript variants have been identified, but the full-length nature of only some of these variants has been described. [provided by RefSeq, Jun 2009]
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UniProt Comments for COL6A3
COL6A3: Collagen VI acts as a cell-binding protein. Defects in COL6A3 are a cause of Bethlem myopathy (BM). BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A3 are a cause of Ullrich congenital muscular dystrophy (UCMD); also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy. Belongs to the type VI collagen family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted; Extracellular matrix

Chromosomal Location of Human Ortholog: 2q37

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; endoplasmic reticulum lumen; extracellular region; collagen type VI; vesicle; sarcolemma

Molecular Function: serine-type endopeptidase inhibitor activity

Biological Process: axon guidance; collagen catabolic process; extracellular matrix disassembly; extracellular matrix organization and biogenesis; muscle development; cell adhesion

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy; Dystonia 27
Research Articles on COL6A3
1. Recessive mutations in the alpha3 (VI) collagen gene COL6A3 cause early-onset isolated dystonia.
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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
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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