The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/100 - 1/500. Detects a band of approximately 175 kDa (predicted molecular weight: 175 kDa).
FunctionMultifunctional 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.
Tissue specificityLiver, 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.
Involvement in diseaseGlycogen storage disease 3
Sequence similaritiesBelongs to the glycogen debranching enzyme family.
Post-translational modificationsThe N-terminus is blocked. Ubiquitinated.
Cellular localizationCytoplasm. Under glycogenolytic conditions localizes to the nucleus.