The exact mechanism by which TANGO2 variants cause pathogenicity is not completely understood. The gene has a dual role in Golgi-to-ER transport as well as oxidation of fatty acids in mitochondria. Transport and Golgi organization (TANGO) proteins were originally characterized n Drosophila, and were thought to play a role in loading newly synthed proteins in the endoplasmic reticulum. Work in T10, the mouse ortholog of TANGO2, has shown it to be robustly expressed in mitochondria enriched fractions of mice brain lysates.
TANGO2