S. Lilling

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A 30-year-old woman with clinical features and biochemical findings of muscle phosphofructokinase deficiency was found to have a very low level of α-1,4-glucan:α-1,4-glucan-6-transglucosylase (branching enzyme, EC 2.4.1.18) activity in muscle. In contrast, branching enzyme activity in the leukocytes was in the range of control values. After sedimentation of(More)
The assay of glycogen debranching activity by measurement of the release of glucose from phosphorylase limit dextrin (PLD), was originally used for assay of enzyme activity in muscle and liver tissue. Results are corrected for the presence of nonspecific glucosidases by subtraction of the activity obtained with glycogen as substrate. When assays are(More)
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