Fruit flies’ diet can help humans find a cure to metabolic diseases
Diet is an important component of our life and is central to keeping an organism healthy. Lack of specific nutrients can result in diseases such as scurvy, in which the lack of vitamin C results in weakness, gum disease, hair loss and bleeding skin. Lack of other nutrients can lead to osteoporosis and rickets (calcium), Beriberi (thiamine), and many other diseases.
Fruit flies can be used to study the impact of nutrition at the cellular level, and to see if by manipulating diet we can improve both normal physiology and complications due to disease. In this study (Sellin et al., 2018) from the laboratory of Margret H. Bulow from the University of Bonn, Germany, Sellin and colleagues explored the impact of lipids on the Zellweger syndrome spectrum, a peroxisome biogenesis disorder which results in newborn babies not living beyond one year of age.
Zellweger syndrome is caused by the malfunctioning peroxisomes, which are a part of the cell responsible for fatty acid digestion and other metabolic functions. The peroxisome machinery in fruit flies is similar to that of humans. Our extensive knowledge of fruit fly genetics and the ease of their genetic manipulation make fruit flies an excellent system to study peroxisome-related diseases. Here, the authors studied a model of Zellweger syndrome that is due to lack of Pex19, a gene required for proper peroxisome formation. The authors found that in this disease model there is accumulation of very long fatty acids, and the important medium length fatty acids were reduced. Dietary supplementation with coconut oil, which contains these important medium chain fatty acids, enabled otherwise non-viable Pex19 mutant larvae to grow and develop into adults, and overall improve their conditions.
The authors further found that this coconut oil supplementation method also corrected cell physiology of a Zellweger patient-derived cell line, thus raising possibilities for new therapies based on dietary supplementation for peroxisomal diseases.
Citation: Sellin, J. et al. (2018) ‘Dietary rescue of lipotoxicity-induced mitochondrial damage in Peroxin19 mutants’, PLOS Biology. Edited by H. Bellen, 16(6), p. e2004893. doi: 10.1371/journal.pbio.2004893.
Fruit flies can be used to study the impact of nutrition at the cellular level, and to see if by manipulating diet we can improve both normal physiology and complications due to disease. In this study (Sellin et al., 2018) from the laboratory of Margret H. Bulow from the University of Bonn, Germany, Sellin and colleagues explored the impact of lipids on the Zellweger syndrome spectrum, a peroxisome biogenesis disorder which results in newborn babies not living beyond one year of age.
Zellweger syndrome is caused by the malfunctioning peroxisomes, which are a part of the cell responsible for fatty acid digestion and other metabolic functions. The peroxisome machinery in fruit flies is similar to that of humans. Our extensive knowledge of fruit fly genetics and the ease of their genetic manipulation make fruit flies an excellent system to study peroxisome-related diseases. Here, the authors studied a model of Zellweger syndrome that is due to lack of Pex19, a gene required for proper peroxisome formation. The authors found that in this disease model there is accumulation of very long fatty acids, and the important medium length fatty acids were reduced. Dietary supplementation with coconut oil, which contains these important medium chain fatty acids, enabled otherwise non-viable Pex19 mutant larvae to grow and develop into adults, and overall improve their conditions.
The authors further found that this coconut oil supplementation method also corrected cell physiology of a Zellweger patient-derived cell line, thus raising possibilities for new therapies based on dietary supplementation for peroxisomal diseases.
Citation: Sellin, J. et al. (2018) ‘Dietary rescue of lipotoxicity-induced mitochondrial damage in Peroxin19 mutants’, PLOS Biology. Edited by H. Bellen, 16(6), p. e2004893. doi: 10.1371/journal.pbio.2004893.