Lysosomal myopathies: an excessive build-up in autophagosomes is too much to handle
MC Malicdan, S Noguchi, I Nonaka, P Saftig… - Neuromuscular …, 2008 - Elsevier
Lysosomes are membrane-bound acidic organelles that contain hydrolases used for
intracellular digestion of various macromolecules in a process generally referred to as
autophagy. In normal skeletal and cardiac muscles, lysosomes usually appear
morphologically unremarkable and thus are not readily visible on light microscopy. In distinct
neuromuscular disorders, however, lysosomes have been shown to be structurally abnormal
and functionally impaired, leading to the accumulation of autophagic vacuoles in myofibers …
intracellular digestion of various macromolecules in a process generally referred to as
autophagy. In normal skeletal and cardiac muscles, lysosomes usually appear
morphologically unremarkable and thus are not readily visible on light microscopy. In distinct
neuromuscular disorders, however, lysosomes have been shown to be structurally abnormal
and functionally impaired, leading to the accumulation of autophagic vacuoles in myofibers …
