TACO1 Human

The Translational Activator of Mitochondrially Encoded Cytochrome C Oxidase, commonly referred to as TACO1, is a mitochondrial protein that plays a crucial role in the translation of mitochondrially-encoded cytochrome c oxidase subunit I (MTCO1). This protein is essential for the proper functioning of the mitochondrial respiratory chain, which is vital for cellular energy production.

The TACO1 gene is located on chromosome 17q23.3 and encodes a protein consisting of 297 amino acids . The protein has an N-terminal mitochondrial presequence of 26 amino acids, which directs it to the mitochondria. The mature protein has a calculated molecular mass of approximately 29.8 kDa . TACO1 is conserved across various species, indicating its fundamental role in cellular metabolism.

TACO1 functions as a translational activator, specifically facilitating the translation of the MTCO1 subunit of cytochrome c oxidase (COX), which is a component of complex IV of the mitochondrial respiratory chain . This complex is responsible for the final step of the electron transport chain, where electrons are transferred to oxygen, resulting in the production of water and the generation of a proton gradient used to produce ATP.

Mutations in the TACO1 gene are associated with mitochondrial complex IV deficiency, which can manifest as Leigh syndrome . Leigh syndrome is a severe neurological disorder characterized by progressive loss of mental and movement abilities. The deficiency in complex IV impairs cellular respiration, leading to reduced ATP production and increased production of reactive oxygen species, which can cause cellular damage.

Research on TACO1 has provided insights into the mechanisms of mitochondrial translation and the pathology of mitochondrial diseases. Understanding the function of TACO1 and its role in mitochondrial translation can aid in the development of therapeutic strategies for conditions associated with mitochondrial dysfunction .