Branching enzyme-deficiency glycogenosis: studies in therapy.

  title={Branching enzyme-deficiency glycogenosis: studies in therapy.},
  author={John Fernandes and F Huijing},
  journal={Archives of disease in childhood},
  volume={43 229},
Type IV glycogen storage disease is caused by a deficiency of the branching enzyme, oc-1,4-glucan: ox-1,4-glucan 6-glucosyltransferase, EC (Brown and Brown, 1966); and is characterized by the accumulation in liver, spleen, and other organs of glycogen with an abnormal structure. This glycogen has long outer chains, relatively few branch points, and gives a blue colour with iodine. It thus resembles amylopectin, and the disease is sometimes called amylopectinosis. The disease is rare… CONTINUE READING
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