The human protein atlas blog

TACO1 an ubiquitous protein governing expression of the mitochondrial genome

Cell Atlas cells Image of the week Immunofluorescence microscopy Mitochondria Mitochondrial disorders

Antibody HPA021643 in U-2 OS recognizing TACO1 in mitochondria.

Mitochondria harbors an own genome that renders key proteins involved in production of energy through oxidation of various substrates. Majority of the disorders associated with mitochondrial function are caused by impaired expression of the proteins encoded in the mitochondrial genome. One of these proteins is the translational activator of cytochrome c oxidase 1 (TACO1).

Experimental results reveal that TACO1 is expressed in all tissues within the human body. The protein is detected mainly in the cytoplasm and more precise inside mitochondria. Explore expression and subcellular localization of TACO1 in the Cell Atlas.

TACO1 is expressed in the cytoplasm and subsequently imported into the mitochondria where it functions as a regulator of protein expression, in particular as a translation activator for the mitochondrial encoded cytochrome c oxidase 1, which is a component of complex IV of the mitochondrial respiratory complex.

Deficiencies associated with this protein complex, which can occur due to mutations in the TACO1 gene for example (Weraarpachai et al., 2009), are a possible cause for the development of Leigh syndrome (Willems JL et al., 1977). The Leigh syndrome is a severe neurodegenerative disorder characterized by a progressive loss of both mental and movement abilities resulting in death within a few years (Dahl et al., 1998).

90% of energy required by the cell is produced with the involvement of the mitochondrion. Loss of mitochondrial function can severely alter biological functions in cells and organs, which inevitably leads to severe disorders highlighted in our previous posts.

Christian Gnann

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