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Biochim Biophys Acta. 2016 Apr;1861(4):269-84. doi: 10.1016/j.bbalip.2016.01.006. Epub 2016 Jan 14.

Autophagy, lipophagy and lysosomal lipid storage disorders.

Author information

1
Institute of Biomedical Research, Institute of Cancer and Genomic Sciences, College of Medical and Dental Sciences, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom.
2
Department of Medicine, Department of Molecular Pharmacology, Institute for Aging Studies, Diabetes Research Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA; Institute for Cell and Molecular Biosciences, Newcastle University Institute for Ageing, Campus for Ageing and Vitality, Newcastle University, Newcastle upon Tyne NE4 5PL, United Kingdom.
3
Institute for Cell and Molecular Biosciences, Newcastle University Institute for Ageing, Campus for Ageing and Vitality, Newcastle University, Newcastle upon Tyne NE4 5PL, United Kingdom.
4
Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, Cambridge, MA 02142, USA.

Abstract

Autophagy is a catabolic process with an essential function in the maintenance of cellular and tissue homeostasis. It is primarily recognised for its role in the degradation of dysfunctional proteins and unwanted organelles, however in recent years the range of autophagy substrates has also been extended to lipids. Degradation of lipids via autophagy is termed lipophagy. The ability of autophagy to contribute to the maintenance of lipo-homeostasis becomes particularly relevant in the context of genetic lysosomal storage disorders where perturbations of autophagic flux have been suggested to contribute to the disease aetiology. Here we review recent discoveries of the molecular mechanisms mediating lipid turnover by the autophagy pathways. We further focus on the relevance of autophagy, and specifically lipophagy, to the disease mechanisms. Moreover, autophagy is also discussed as a potential therapeutic target in several key lysosomal storage disorders.

KEYWORDS:

Autophagy; Lipid metabolism; Lipid storage disorders

PMID:
26778751
DOI:
10.1016/j.bbalip.2016.01.006
[Indexed for MEDLINE]
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