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Garrido-Maraver 2019 Cell Death Dis

From Bioblast
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Publications in the MiPMap
Garrido-Maraver J, Celardo I, Costa AC, Lehmann S, Loh SHY, Martins LM (2019) Enhancing folic acid metabolism suppresses defects associated with loss of Drosophila mitofusin. Cell Death Dis 10:288.

Β» PMID: 30911005 Open Access

Garrido-Maraver J, Celardo I, Costa AC, Lehmann S, Loh SHY, Martins LM (2019) Cell Death Dis

Abstract: Mutations in the mitochondrial GTPase mitofusin 2 (MFN2) cause Charcot-Marie-Tooth disease type 2 (CMT2A), a form of peripheral neuropathy that compromises axonal function. Mitofusins promote mitochondrial fusion and regulate mitochondrial dynamics. They are also reported to be involved in forming contacts between mitochondria and the endoplasmic reticulum. The fruit fly, Drosophila melanogaster, is a powerful tool to model human neurodegenerative diseases, including CMT2A. Here, we have downregulated the expression of the Drosophila mitofusin (dMfn RNAi) in adult flies and showed that this activates mitochondrial retrograde signalling and is associated with an upregulation of genes involved in folic acid (FA) metabolism. Additionally, we demonstrated that pharmacological and genetic interventions designed to increase the FA metabolism pathway suppresses the phenotype of the dMfn RNAi flies. We conclude that strategies to increase FA metabolism may ameliorate diseases, such as peripheral neuropathies, that are associated with loss of mitochondrial function. A video abstract for this article is available at https://youtu.be/fs1G-QRo6xI.

β€’ Bioblast editor: Plangger M β€’ O2k-Network Lab: UK Leicester Martins LM


Labels: MiParea: Respiration, Genetic knockout;overexpression, Pharmacology;toxicology  Pathology: Neurodegenerative 

Organism: Drosophila 

Preparation: Permeabilized tissue 


Coupling state: OXPHOS, ET  Pathway: N, S  HRR: Oxygraph-2k 

Labels, 2019-04