The importance of quality control of mitochondrial translation is revealed for the first time.

Recently, Wang Endo Ofor Ofdor Research Group, of the Institute of Biochemistry and Cell Biology of the Shanghai Institute of Life Sciences of the Chinese Academy of Sciences, in collaboration with Finnish scientists, published in nucleic acid research entitled Editing Activity for the fielding mischarged tRNAs is essential in mammalian mitochondria.
mammalian cells contain two relatively independent translation systems: cytoplasm and mitochondrial translation systems.
human mitochondrial translation system synthesizes 13 mitochondrial genome-coded oxidizing respiratory chain complex subunits, which are very important for the assembly of oxidation respiratory chain complexes and the function of mitochondrial.
cytoplasm translation systemrequires a high degree of fidelity to ensure the precise transfer of nuclear genetic information.
, for example, the absence or impairment of fidelity of the mouse cytoplasmic propylene-tRNA synth asse (alanyl-tRNA synthetase, AlaRS) causes the wrong protein to focus, which in turn can lead to neurodegenerative disease or heart disease.
little is known about the fidelity of mitochondrial genome transmission and its invivia.
the first time in the study, researchers cloned and expressed a mature form of human mitochondrial protoplasm-tRNA synth (hmalAlaRS) gene AARS2, purifying the high-purity hmtAlaRS.
in vitro studies showed that hmtAlaRS significantly misactivated the non-corresponding amino acid Ser, the use of post-transfer editing reaction hydrolysis misbutyled aminoized product Ser-tRNAAla;
further obtained mouse mitochondrial AlaRS (mmtAlaRS) C744A (corresponding to hmtAlaRS C749A) point mutant mice and V755E (corresponding to hmtAlaRS V760E) point mutant mice, found that pure mouse embryos died, confirmed that the mtAlaRS-mediated impaired reaction to the normal function of mitochondrial mice and the development of an important role.
the study reveals the importance of quality control of mitochondrial translation for the first time at animal level.
research work has been supported by the National Key Research and Development Program, the National Natural Science Foundation, the Chinese Academy of Sciences, the Shanghai Science and Technology Commission, etc.
.