Role of Mutations of Mitochondrial Aminoacyl-tRNA Synthetases Genes on Epileptogenesis

Mitochondria are the structures in cells that are responsible for producing energy. They contain a specific translation unit for synthesizing mitochondria-encoded respiratory chain components: the mitochondrial DNA (mt DNA). Recently, a growing number of syndromes associated with the dysfunction of...

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Bibliographic Details
Published in:Molecular neurobiology 2023-09, Vol.60 (9), p.5482-5492
Main Authors: Kong, Ling-yue, Wu, Yi-ze, Cheng, Run-qi, Wang, Pei-han, Peng, Bi-wen
Format: Article
Language:English
Subjects:
Adenosine triphosphate
Alzheimer's disease
Amino acids
Aminoacyl-tRNA ligase
Apoptosis
Biomedical and Life Sciences
Biomedicine
Biosynthesis
Cell Biology
DNA methylation
Drug resistance
Electron transport
Energy
Enzymes
Epilepsy
Genes
Genomes
Genomics
Kinases
Metabolism
Mitochondria
Mitochondrial DNA
Mutation
Nervous system
Neurobiology
Neurology
Neurosciences
Phosphorylation
Physiology
Protein synthesis
Proteins
Transfer RNA
Translation
tRNA
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Summary:Mitochondria are the structures in cells that are responsible for producing energy. They contain a specific translation unit for synthesizing mitochondria-encoded respiratory chain components: the mitochondrial DNA (mt DNA). Recently, a growing number of syndromes associated with the dysfunction of mt DNA translation have been reported. However, the functions of these diseases still need to be precise and thus attract much attention. Mitochondrial tRNAs (mt tRNAs) are encoded by mt DNA; they are the primary cause of mitochondrial dysfunction and are associated with a wide range of pathologies. Previous research has shown the role of mt tRNAs in the epileptic mechanism. This review will focus on the function of mt tRNA and the role of mitochondrial aminoacyl-tRNA synthetase (mt aaRS) in order to summarize some common relevant mutant genes of mt aaRS that cause epilepsy and the specific symptoms of the disease they cause.
ISSN:0893-7648
1559-1182
DOI:10.1007/s12035-023-03429-1