Full Product Name
Human AGL Polyclonal Antibody
Product Synonym Names
GDE; Glycogen debrancher
Product Gene Name
anti-AGL antibody
[Similar Products]
Matching Pairs
Antibody: AGL (MBS2897552)
Antigen: Glycogen debranching enzyme (MBS2889266)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P35573
Purity/Purification
Immunogen Affinity Purified
Form/Format
Liquid; 0.1MxPBS, 50% Glycerol, pH7.5
Concentration
200 ug/ml (lot specific)
Immunogen
Recombinant Human AGL Protein
Preparation and Storage
Store at 4 degree C for frequent use. Store at -20 degree C to -70 degree C for 6 months.
Other Notes
Small volumes of anti-AGL antibody 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.
Applications Tested/Suitable for anti-AGL antibody
Western Blot (WB), Immunohistochemistry (IHC)
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 #
NP_000019.2
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UniProt Primary Accession #
P35573
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UniProt Related Accession #
P35573[Other Products]
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]
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; EC 2.4.1.25; EC 3.2.1.33; Hydrolase; Transferase; Ubiquitin conjugating system
Chromosomal Location of Human Ortholog: 1p21.2
Cellular Component: cytoplasm; cytosol; extracellular region; ficolin-1-rich granule lumen; inclusion body; isoamylase complex; nucleus; sarcoplasmic reticulum; secretory granule lumen
Molecular Function: 4-alpha-glucanotransferase activity; amylo-alpha-1,6-glucosidase activity; glycogen debranching enzyme activity; polysaccharide binding; polyubiquitin modification-dependent protein binding; protein binding
Biological Process: glycogen biosynthetic process; glycogen catabolic process; neutrophil degranulation; response to glucocorticoid stimulus; response to nutrient
Disease: Glycogen Storage Disease Iii
Research Articles on AGL
1. This report of patients with GSD-III in Iran with 2 uncommon clinical presentations and 5 novel mutations in the AGL gene.
Precautions
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