Anti-LARS2 antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

LARS2 also known as mitochondrial leucyl-tRNA synthetase 2 plays a significant role in protein biosynthesis. This enzyme is part of the class II aminoacyl-tRNA synthetase family with a molecular mass of around 108 kDa. LARS2 primarily functions in mitochondria ensuring the proper attachment of leucine to its corresponding tRNA an essential step for protein translation. Expression of LARS2 occurs most notably in tissues with high metabolic demands such as muscle and liver where mitochondrial activity is important.

Biological function summary

LARS2 facilitates the translation of mitochondrial-encoded proteins by charging tRNA with leucine. It operates as a monomer and does not participate in a larger complex. This charging process is essential for maintaining mitochondrial function and energy production ensuring the correct synthesis of respiratory chain components. LARS2 thereby provides support for the translation machinery within mitochondria.

Pathways

LARS2 plays an indispensable role within the mitochondrial translation system. It directly influences the oxidative phosphorylation pathway by enabling the synthesis of components important for electron transport and ATP production. LARS2 interacts functionally with other mitochondrial tRNA synthetases like GARS2 and YARS2 contributing to the efficient translation of proteins fundamental for cellular respiration.

Associated diseases and disorders

Mutations in the LARS2 gene have been linked to Perrault syndrome a condition characterized by ovarian dysgenesis and sensorineural deafness. Additionally disruptions in LARS2 activity may also contribute to mitochondrial encephalopathy. The dysfunction of LARS2 affects proteins within the mitochondrial oxidative phosphorylation pathway impacting ATP production and cellular energy balance which further associates with disorders involving METTL1 and other mitochondrial synthetases impacting similar processes.