Product Name
AGL, Blocking Peptide
Product Synonym Names
Glycogen debranching enzyme; Glycogen debrancher; 4-alpha-glucanotransferase; Oligo-1;4-1;4-glucantransferase; Amylo-alpha-1;6-glucosidase; Amylo-1;6-glucosidase; Dextrin 6-alpha-D-glucosidase; AGL; GDE
Product Gene Name
AGL blocking peptide
[Similar Products]
Product Synonym Gene Name
GDE[Similar Products]
Antibody/Peptide Pairs
AGL peptide (MBS9224655) is used for blocking the activity of AGL antibody (MBS9217121)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P35573
Specificity
The synthetic peptide sequence used to generate the antibody was selected from the N-term region of human AGL. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Form/Format
The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.
Cellular Location
Cytoplasm. Note: Under glycogenolytic conditions localizes to the nucleus
Tissue Location
Liver, kidney and lymphoblastoid cells express predominantly isoform 1; whereas muscle and heart express not only isoform 1, but also muscle-specific isoform mRNAs (isoforms 2, 3 and 4). Isoforms 5 and 6 are present in both liver and muscle
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of AGL blocking peptide 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 blocking peptide
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.
NCBI/Uniprot data below describe general gene information for AGL. It may not necessarily be applicable to this product.
NCBI Accession #
P35573.3
[Other Products]
UniProt Primary Accession #
P35573
[Other Products]
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
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: Carbohydrate Metabolism - starch and sucrose; Hydrolase; Ubiquitin conjugating system; EC 3.2.1.33; EC 2.4.1.25; Transferase
Chromosomal Location of Human Ortholog: 1p21
Cellular Component: cytoplasm; cytosol; isoamylase complex; nucleus
Molecular Function: 4-alpha-glucanotransferase activity; amylo-alpha-1,6-glucosidase activity; glycogen debranching enzyme activity; protein binding
Biological Process: glycogen catabolic process
Disease: Glycogen Storage Disease Iii
Research Articles on AGL
1. Point mutations in AGL gene are associated with glycogen storage disease type IIIa in a Chinese family.
Precautions
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Disclaimer
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