Abstract
Pathological phenotypes in inclusion body myopathy (IBM) associated with Paget disease of the bone (PDB), frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) (IBMPFD/ALS) include defective autophagosome and endosome maturation that result in vacuolation, weakness and muscle atrophy. The link between autophagy and IBMPFD/ALS pathobiology has been poorly understood. We examined the AKT-FOXO3 and MTOR pathways to characterize the regulation of autophagy in IBMPFD/ALS mouse muscle. We identified a defect in MTOR signaling that results in enhanced autophagosome biogenesis. Modulating MTOR signaling may therefore be a viable therapeutic target in IBMPFD/ALS.
Original language | English |
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Pages (from-to) | 799-800 |
Number of pages | 2 |
Journal | Autophagy |
Volume | 9 |
Issue number | 5 |
DOIs | |
State | Published - May 2013 |
Keywords
- Autophagy
- MTOR
- Myopathy
- Rapamycin
- VCP