Open AccessFurther Characterization of Glycogen from Type‐IV Glycogen‐Storage Disease
Author(s) -
Mercier Christiane,
Whelan William J.
Publication year - 1973
Publication title -
european journal of biochemistry
Language(s) - English
Resource type - Journals
eISSN - 1432-1033
pISSN - 0014-2956
DOI - 10.1111/j.1432-1033.1973.tb03189.x
Subject(s) - isoamylase , amylopectin , glycogen , glycogen debranching enzyme , glycogen storage disease , chemistry , biochemistry , medicine , biology , glycogen synthase , starch , amylose , enzyme , amylase
Glycogen from a male child suffering from type IV glycogenosis was debranched with bacterial isoamylase and fractionated on Sephadex G‐50. The unit chains, set free by the isoamylase, emerged as a single peak, similar to that for normal human liver glycogen, but of higher average chain length. The pattern of the unit chains was distinctly different from that shown by amylopectin, which displays two populations of unit chains. It is thereby reaffirmed that, contrary to previous belief, type IV glycogen is not amylopectin‐like in structure, and the name “amylopectinosis” for this disease is inappropriate.