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Crystal structures of Escherichia coli branching enzyme in complex with cyclodextrins.
The definitive diagnosis rests on the demonstration of the deficiency of branching enzyme activity in liver, muscle, cultured skin fibroblasts, or leukocytes.
Also, the flux control coefficients of other enzymes in the pathway of sugar and starch metabolism in cereal grains, such as branching enzyme, AGPase, and sucrose synthase (Singletary et al., 1997) are much lower than that reported for SSS (Hawker and Jenner, 1993; Jenner et al., 1993; Keeling et al., 1993).