Full Product Name
GCSH siRNA (Human)
Product Synonym Names
Glycine cleavage system H protein mitochondrial; Lipoic acid-containing protein
Product Gene Name
GCSH sirna
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P23434
Specificity
GCSH 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 GCSH 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 GCSH 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
GCSH sirna
siRNA to inhibit GCSH expression using RNA interference
Applications Tested/Suitable for GCSH sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for GCSH. It may not necessarily be applicable to this product.
NCBI Accession #
NP_004474.2
[Other Products]
NCBI GenBank Nucleotide #
NM_004483.4
[Other Products]
UniProt Primary Accession #
P23434
[Other Products]
UniProt Secondary Accession #
Q9H1E9[Other Products]
UniProt Related Accession #
P23434[Other Products]
Molecular Weight
18,885 Da
NCBI Official Full Name
glycine cleavage system H protein, mitochondrial
NCBI Official Synonym Full Names
glycine cleavage system protein H (aminomethyl carrier)
NCBI Official Symbol
GCSH [Similar Products]
NCBI Official Synonym Symbols
GCE; NKH
[Similar Products]
NCBI Protein Information
glycine cleavage system H protein, mitochondrial
UniProt Protein Name
Glycine cleavage system H protein, mitochondrial
UniProt Synonym Protein Names
Lipoic acid-containing protein
Protein Family
Glycine cleavage system H-like protein
UniProt Gene Name
GCSH [Similar Products]
UniProt Entry Name
GCSH_HUMAN
NCBI Summary for GCSH
Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.[provided by RefSeq, Jan 2010]
UniProt Comments for GCSH
GCSH: The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvH family.
Chromosomal Location of Human Ortholog: 16q23.2
Cellular Component: mitochondrion; glycine cleavage complex
Molecular Function: enzyme binding; aminomethyltransferase activity
Biological Process: methylation; glycine decarboxylation via glycine cleavage system; glycine catabolic process
Disease: Glycine Encephalopathy
Research Articles on GCSH
1. There is no detectable glycine cleavage enzyme activity in human skin fibroblasts.
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.