Journal
AUTOPHAGY
Volume 5, Issue 6, Pages 795-804Publisher
TAYLOR & FRANCIS INC
DOI: 10.4161/auto.8901
Keywords
autophagy; nuclear envelopathies; A-type lamins; emerin; nucleus
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Funding
- Research oil Psychiatric and Neurological Diseases and Mental Health of Health Labour Sciences Research Grant
- Ministry of Health, Labor and Welfare [20B-12, 20B-13]
- Human Frontier Science Program
- Japan Society for the Promotion of Science
- Japanese Health Sciences Foundation
- Fundamental Studies in Health Sciences of the National Institute of Biomedical Innovation (NIBIO)
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Autophagy is an evolutionarily conserved intracellular mechanism for the degradation of organelles and proteins. Here we demonstrate the presence of perinuclear autophagosomes/autolysosomes containing nuclear components in nuclear envelopathies caused by mutations in the genes encoding A-type lamins (LMNA) and emerin (EMD). These autophagosomes/autolysosomes were sometimes bigger than a nucleus. The autophagic nature is further supported by upregulation of LC3-II in Lmna(H222P/H222P) fibroblasts. In addition, inhibition of autophagy led to the accumulation of nuclear abnormalities and reduced cell viability, strongly suggesting a beneficial role of autophagy, at least in these cells. Similar giant autophagosomes/autolysosomes were seen even in wild-type cells, albeit rarely, implying that this nucleophagy' is not confined to the diseased condition, but may be seen even in physiologic conditions to dean up nuclear wastes produced by nuclear damage.
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