Social Science Research Network | 2021
Mitochondrial RBP Complex AMG-1/SLRP-1 is Required for Germline Development and Reproductive Success in Caenorhabditis elegans
Abstract
Mitochondria with their own genome (mtDNA) are the powerhouse for many biological processes. The post-transcriptional regulation of mtDNA is conducted by nuclear-encoded mitochondrial RNA-binding proteins (mtRBPs). However, only a few mtRBPs have been characterized.\xa0Here, we identified an mtRBP AMG-1\xa0(Abnormal Mitochondria in Germline) which is crucial for the reproductive development especially spermatogenesis in\xa0C. elegans. amg-1\xa0mutation caused mitochondrial dysfunction associated with structure defects and disturbed mtDNA transcription in germ cells. Furthermore, AMG-1 deficiency triggers sperm apoptosis and gonadal mitochondrial unfolded protein response non-cell autonomously.\xa0SLRP-1 with RNA recognition motif interacts with AMG-1 genetically to regulate germ cell development.\xa0AMG-1 is\xa0an ortholog of\xa0mammalian LRPPRC,\xa0whose mutations cause\xa0the\xa0French-Canadian type of Leigh syndrome (FCLS)\xa0characterized by neurodegeneration.\xa0Our findings that mtRBP is involved in spermatogenesis by post-transcriptionally regulating mtDNA might\xa0provide implications for understanding of the pathogenic mechanisms of\xa0FCLS and for the development of mitochondrial medicine.