Neuron-specific knockdown of solute carrier protein SLC25A46a induces locomotive defects, an abnormal neuron terminal morphology, learning disability, and shortened lifespan

IBRO Reports
Md Saheb AliMasamitsu Yamaguchi

Abstract

Various mutations in the SLC25A46 gene have been reported in mitochondrial diseases that are sometimes classified as type 2 Charcot-Marie-Tooth disease, optic atrophy, and Leigh syndrome. Although human SLC25A46 is a well-known transporter that acts through the mitochondrial outer membrane, the relationship between neurodegeneration in these diseases and the loss-of-function of SLC25A46 remains unclear. Two Drosophila genes, CG8931 (dSLC25A46a) and CG5755 (dSLC25A46b) have been identified as candidate homologs of human SLC25A46. We previously characterized the phenotypes of pan-neuron-specific dSLC25A46b knockdown flies. In the present study, we developed pan-neuron-specific dSLC25A46a knockdown flies and examined their phenotypes. Neuron-specific dSLC25A46a knockdown resulted in reduced mobility in larvae as well as adults. An aberrant morphology for neuromuscular junctions (NMJs), such as a reduced synaptic branch length and decreased number and size of boutons, was observed in dSLC25A46a knockdown flies. Learning ability was also reduced in the larvae of knockdown flies. In dSLC25A46a knockdown flies, mitochondrial hyperfusion was detected in NMJ synapses together with the accumulation of reactive oxygen species and reductio...Continue Reading

Citations

Aug 28, 2020·International Journal of Molecular Sciences·Rosita CurcioVincenza Dolce
Jan 10, 2021·International Journal of Molecular Sciences·Masamitsu YamaguchiHideki Yoshida
Oct 15, 2020·International Journal of Molecular Sciences·Fukiko Kitani-Morii, Yu-Ichi Noto
Apr 5, 2021·Experimental Cell Research·Masamitsu YamaguchiHideki Yoshida

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Methods Mentioned

BETA
transgenic
PCR
electrophoresis

Software Mentioned

GraphPad Prism
UniProt
FASTA
MetaMorph
ImageJ
BLAST

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