Polyglucosan storage myopathies

C Hedberg-Oldfors, A Oldfors - Molecular aspects of medicine, 2015 - Elsevier
Polyglucosan is an amylopectin-like polysaccharide associated with defective glycogen
metabolism and, unlike normal glycogen, it is to some extent resistant to α-amylase
digestion. It also has a characteristic fibrillar appearance under the electron microscope.
Polyglucosan may aggregate into dense inclusions known as polyglucosan bodies. Its
accumulation can be found in various tissues to some degree in normal ageing, but it is also
the hallmark of some diseases associated with defects in glycogen metabolism. These …