Rabbit polyclonal antibody to EF~Tu (L338)

EF~Ts, also known as TSFM (Ts translation elongation factor, mitochondrial) or COXPD3, is a 325 amino acid protein that is one of 13 mitochondrial~encoded proteins that work together during the elongation phase of protein biosynthesis on the ribosome. Expressed ubiquitously with highest levels present in liver, kidney and skeletal muscle, EF~Ts associates with EF~Tu, a multidomain GTPase with essential functions in translation, and, via this interaction, facilitates the exchange of GDP for GTP, thereby inducing protein elongation. Mutations in the gene encoding EF~Ts are the cause of combined oxidative phosphorylation deficiency type 3 (COXPD3), a condition characterized by defects in the mitochondrial oxidative phosphorylation system and often characterized by severe metabolic acidosis with encephalomyopathy or with hypertrophic cardiomyopathy. Multiple isoforms of EF~Ts exist due to alternative splicing events.