AGL siRNA (Human)

Referência MBS827697-15nmol

Tamanho : 15nmol

Marca : MyBiosource


AGL siRNA – Glycogen Debranching Enzyme Isoform 1 (Human)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS827697

MBS827697 is an siRNA targeting AGL. Also known as AGL siRNA (Human). Reactive in Human. Host: Synthetic. siRNA to inhibit AGL expression using RNA interference More details

Product Overview

Online Datasheet in PDF
Product Name
AGL, siRNA
Full Product Name
AGL siRNA (Human)
Product Gene Name
AGL sirna
Product Synonym Names
GDE; Glycogen debranching enzyme; Glycogen debrancher
Host
Synthetic
Species Reactivity
Human
Form/Format
Lyophilized powder
Purity/Purification
> 97%
Specificity
AGL siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
Preparation and Storage
Shipped at 4 degree C. Store at -20 degree C for one year.
Molecular Weight
172,696 Da
OMIM
232400
3D Structure
ModBase 3D Structure for P35573
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.
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.
Applications Tested/Suitable for AGL sirna
RNA Interference (RNAi)

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.

Additional Details

NCBI/Uniprot data below describe general gene information for AGL. It may not necessarily be applicable to this product.

NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
GenBank Nucleotide #
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
NCBI Official Synonym Symbols
GDE
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
UniProt Synonym Gene Names
GDE; Amylo-1,6-glucosidase
UniProt Entry Name
GDE_HUMAN
NCBI Protein Information
glycogen debranching enzyme

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]
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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
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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.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with AGL sirna

 Reference Product  PubMed Publications
 PYGM sirna  >2 publications with AGL and PYGM
 GBE1 sirna  >2 publications with AGL and GBE1
 GYS2 sirna  >1 publications with AGL and GYS2
 GYS1 sirna  >1 publications with AGL and GYS1
 PYGL sirna  >1 publications with AGL and PYGL

Pathways associated with AGL sirna

 Products by Pathway  Pathway Diagram
 Glucose Metabolism Pathway antibodies  Glucose Metabolism Pathway Diagram
 Glycogen Metabolism Pathway antibodies  Glycogen Metabolism Pathway Diagram
 Glycogen Breakdown (glycogenolysis) Pathway antibodies  Glycogen Breakdown (glycogenolysis) Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Carbohydrates Pathway antibodies  Metabolism Of Carbohydrates Pathway Diagram
 Starch And Sucrose Metabolism Pathway antibodies  Starch And Sucrose Metabolism Pathway Diagram
 Starch And Sucrose Metabolism Pathway antibodies  Starch And Sucrose Metabolism Pathway Diagram

Diseases associated with AGL sirna

 Disease Name  Pubmed Publications
 Glycogen Storage Disease Type III Antibodies  >55 publications with AGL and Glycogen Storage Disease Type III
 Nervous System Diseases Antibodies  >27 publications with AGL and Nervous System Diseases
 Hepatomegaly Antibodies  >20 publications with AGL and Hepatomegaly
 Liver Diseases Antibodies  >20 publications with AGL and Liver Diseases
 Neurobehavioral Manifestations Antibodies  >7 publications with AGL and Neurobehavioral Manifestations
 Disease Models, Animal Antibodies  >7 publications with AGL and Disease Models, Animal
 Inflammation Antibodies  >7 publications with AGL and Inflammation
 Hypertrophy Antibodies  >6 publications with AGL and Hypertrophy
 Kidney Diseases Antibodies  >6 publications with AGL and Kidney Diseases
 Neoplasms, Experimental Antibodies  >6 publications with AGL and Neoplasms, Experimental

Organs/Tissues associated with AGL sirna

 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >61 publications with AGL and Blood
 Brain Antibodies  >40 publications with AGL and Brain
 Muscle Antibodies  >40 publications with AGL and Muscle
 Liver Antibodies  >37 publications with AGL and Liver
 Eye Antibodies  >14 publications with AGL and Eye
 Kidney Antibodies  >11 publications with AGL and Kidney
 Heart Antibodies  >10 publications with AGL and Heart
 Bone Marrow Antibodies  >9 publications with AGL and Bone Marrow
 Skin Antibodies  >5 publications with AGL and Skin
 Lymph Node Antibodies  >3 publications with AGL and Lymph Node