9.B.467.  The Transport and Golgi Organization Protein 2 (TANGO) Family 

Loss of TANGO2 in humans precipitates metabolic crises during periods of heightened energy demand, such as fasting, infections, or high fever. TANGO2 has been implicated in various functions, including lipid metabolism and heme transport. It localizes to the mitochondrial lumen via a structural region containing LIL residues. Mutations in these LIL residues cause TANGO2 to relocate to the periphery of lipid droplets. Purified TANGO2 binds acyl-coenzyme A, and mutations in the highly conserved NRDE sequence of TANGO2 inhibit this binding (Lujan et al. 2025).