|Other 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|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP2402c 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.|
|Format||Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||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.|
|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|
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Provided below are standard protocols that you may find useful for product applications.
AGL is a 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 the AGL gene are associated with glycogen storage disease although a wide range of enzymatic and clinicalvariability occurs which may be due to tissue-specific alternative splicing.
Horinishi, A., et al., J. Hum. Genet. 47(2):55-59 (2002).Shen, J., et al., Hum. Mutat. 9(1):37-40 (1997).Bao, Y., et al., Genomics 38(2):155-165 (1996).Shen, J., et al., J. Clin. Invest. 98(2):352-357 (1996).Yang, B.Z., et al., J. Biol. Chem. 267(13):9294-9299 (1992).
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