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AGL

amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase

Glycogen debranching enzyme is involved in glycogen degradation and has two independent catalytic activities: a 4-alpha-glucotransferase activity (EC 2.4.1.25) and a amylo-1,6-glucosidase activity (EC 3.4.1.33). Both activities occur at different sites on the single polypeptide chain. Mutations in this gene cause glycogen storage disease. A wide range of clinical and enzymatic variability occurs in glycogen debrancher deficiency, some of which may be due to tissue-specific alternative splicing. Six splice variants that differ in the 5' end have been identified in liver and muscle tissue. Variants 1, 5, and 6 are present in both liver and muscle, whereas variants 2, 3, and 4 occur in muscle. Variants 1 through 4 encode identical proteins (isoform 1) that include 27 N-terminal amino acids not found in splice variants 5 and 6. Variants 5 and 6 encode different amino-terminal ends of 10 and 11 amino acids in protein isoforms 2 and 3, respectively, with the remainder of the peptide identical to that of isoforms 1.

Gene Name: amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
Synonyms: AGL, GDE, Glycogen debrancher, Glycogen debranching enzyme
Target Sequences: NM_000028 NP_000019.2 P35573

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ELISA Kits (3)
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AGL ELISA Kit
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Sandwich
96-Well Microplate
Human
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Cell Lysates, Tissue Homogenates
1 Plate/$730
AGL ELISA Kit
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Custom
96-Well Strip Plate
Dog
Colorimetric - 450nm (TMB)
1 Plate/$783
AGL ELISA Kit
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Sandwich
96-Well Strip Plate
Human
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
Plasma, Serum
1 Plate/$669
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PLEASE NOTE

For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).