Polyglucosan (PG) is an abnormal polysaccharide that, compared to glycogen, has fewer branched points and excessively long peripheral chains that structurally resemble the plant polysaccharide "amylopectin". Under electron microscopy, PG bodies are round, non-membrane-bound cytoplasmic particles with irregular branched filaments, which often displace myofibrils, leading to Z disk streaming. PG bodies react strongly to PAS stain and are partially resistant to diastase digestion.

Fanin M, Nascimbeni A, Savarese M, Papa V, Cenacchi G, Nigro V, et al. (2015). Familial Polyglucosan Body Myopathy with Unusual Phenotype. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 38(5), 295-302 [10.1111/nan.12171].

Familial Polyglucosan Body Myopathy with Unusual Phenotype

PAPA, VALENTINA;CENACCHI, GIOVANNA;
2015

Abstract

Polyglucosan (PG) is an abnormal polysaccharide that, compared to glycogen, has fewer branched points and excessively long peripheral chains that structurally resemble the plant polysaccharide "amylopectin". Under electron microscopy, PG bodies are round, non-membrane-bound cytoplasmic particles with irregular branched filaments, which often displace myofibrils, leading to Z disk streaming. PG bodies react strongly to PAS stain and are partially resistant to diastase digestion.
2015
Fanin M, Nascimbeni A, Savarese M, Papa V, Cenacchi G, Nigro V, et al. (2015). Familial Polyglucosan Body Myopathy with Unusual Phenotype. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 38(5), 295-302 [10.1111/nan.12171].
Fanin M; Nascimbeni A; Savarese M; Papa V; Cenacchi G; Nigro V; Angelini C
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/378268
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