MT-RNR2 and phenylketonuria: l-DOPA, the product of Tyr catalyzed by tyrosine hydroxylase, was also effective in improving Hn knockdown Drosophila (Fig. 1I; Fig. S7D), illustrating that Tyr and l-DOPA shortages are the key defective elements in the PKU Drosophila. In Drosophila, the two closest homologs of human LAT1/SLC7A5, the L-type amino acid transporter responsible for the uptake of large neutral amino acids, are CG1607 and CG12317. Only CG1607 RNAi dramatically decreased the eclosion rate of Hn RNAi Drosophila in the Phe food (Fig. 1J).