Full Product Name
AGL siRNA (Human)
Product Synonym Names
GDE; Glycogen debranching enzyme; Glycogen debrancher
Product Gene Name
AGL sirna
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P35573
Specificity
AGL 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 AGL 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 AGL 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
AGL sirna
siRNA to inhibit AGL expression using RNA interference
Applications Tested/Suitable for AGL sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for AGL. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000019.2
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NCBI GenBank Nucleotide #
NM_000028.2
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UniProt Primary Accession #
P35573
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UniProt Secondary Accession #
P78354; P78544; Q59H92; Q6AZ90; Q9UF08; A6NCX7; A6NEK2; D3DT51[Other Products]
UniProt Related Accession #
P35573[Other Products]
Molecular Weight
172,696 Da
NCBI Official Full Name
glycogen debranching enzyme isoform 1
NCBI Official Synonym Full Names
amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
NCBI Official Symbol
AGL??[Similar Products]
NCBI Official Synonym Symbols
GDE
??[Similar Products]
NCBI Protein Information
glycogen debranching enzyme
UniProt Protein Name
Glycogen debranching enzyme
UniProt Synonym Protein Names
Glycogen debrancherIncluding the following 2 domains:4-alpha-glucanotransferase (EC:2.4.1.25)Alternative name(s):Oligo-1,4-1,4-glucantransferase
UniProt Gene Name
AGL??[Similar Products]
UniProt Synonym Gene Names
GDE; Amylo-1,6-glucosidase??[Similar Products]
UniProt Entry Name
GDE_HUMAN
NCBI Summary for AGL
This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
UniProt Comments for AGL
GDE: Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4- alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6- glucosidase in glycogen degradation. Defects in AGL are the cause of glycogen storage disease type 3 (GSD3); also known as Forbes disease. GSD3 is a metabolic disorder associated with an accumulation of abnormal glycogen with short outer chains. Three GSD3 types are recognized: GSD type 3A patients lack glycogen debrancher enzyme activity in both liver and muscle, while GSD type 3B patients are enzyme- deficient in liver only. In rare cases, selective loss of only 1 of the 2 debranching activities, glucosidase or transferase, results in GSD type 3C or type 3D, respectively. GSD3 is clinically characterized by hepatomegaly, hypoglycemia, short stature, and variable myopathy. Belongs to the glycogen debranching enzyme family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.2.1.33; Ubiquitin conjugating system; Carbohydrate Metabolism - starch and sucrose; Hydrolase; Transferase; EC 2.4.1.25
Chromosomal Location of Human Ortholog: 1p21
Cellular Component: nucleoplasm; sarcoplasmic reticulum; cytoplasm; isoamylase complex; inclusion body; cytosol
Molecular Function: protein binding; glycogen debranching enzyme activity; 4-alpha-glucanotransferase activity; polyubiquitin binding; amylo-alpha-1,6-glucosidase activity; polysaccharide binding
Biological Process: glycogen biosynthetic process; glycogen catabolic process; response to glucocorticoid stimulus; carbohydrate metabolic process; glucose metabolic process; pathogenesis; response to nutrient
Disease: Glycogen Storage Disease Iii
Research Articles on AGL
1. A homozygous frameshift deletion, c.4456delT, in exon 33 of the AGL gene in Inuit children determines the cause of glycogen storage disease type IIIa and confirms a founder effect.
Precautions
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Disclaimer
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