Cysteinyl-tRNA synthetase 2, mitochondrial (CARS2)

Cysteinyl-tRNA synthetase 2, mitochondrial (CARS2) is a mitochondrial-specific member of the aminoacyl-tRNA synthetase (ARS) family, responsible for attaching cysteine to its corresponding tRNA within mitochondria[2]. In addition to its canonical translational role, CARS2 possesses "moonlighting" activity as a cysteine persulfide synthase (CPERS), which supports the production of cysteine persulfides involved in the regulation of protein polysulfidation, mitochondrial biogenesis, morphology, and energy metabolism including electron transport chain activity[1][3]. CARS2 is essential for mitochondrial function and localizes to mitochondria via N-terminal targeting signals; its deficiency leads to marked changes in mitochondrial structure, reduced biogenesis, impaired membrane potential, and defective bioenergetics[1][2]. Loss-of-function mutations in CARS2 are associated with combined oxidative phosphorylation deficiency 27 (COXPD27), a mitochondrial disease[4]. CARS2 is not currently known to interact with approved drugs, nor is it used as a routine biomarker or therapeutic target, but its essential role makes it a potential candidate for further study in mitochondrial and metabolic disorders. Targeting or modifying CARS2 activity poses notable safety risks due to its fundamental function in protein translation and energy metabolism.

Other names

Probable cysteine--tRNA ligase, mitochondrialOK/SW-cl.10CysRSFLJ12118Cysteinyl-tRNA synthetasecysteine tRNA ligase 2, mitochondrialCOXPD27cysRSputative cysteine--tRNA ligase, mitochondrialcysteinyl-tRNA synthetase 2, mitochondrial (putative)