Heart mitochondria metabolize 3-methylthiopropionate to CO2 and methanethiol.

3-Methylthiopropionate (MTP) stimulates respiration of substrate-depleted heart mitochondria. This is blocked by uncouplers and by malonate. With the use of methyl-14C- and uniformly 14C-labeled MTP, it was found that methanethiol and CO2 are reaction products. The methyl carbon was not significantly oxidized. This study, together with a recent report [P. W. D. Scislowski et al. (1987) Biochem. J. 247, 35-40], demonstrates the existence of a transsulfuration independent pathway of methionine metabolism by muscle, and that the complete pathway following the initial transamination is a mitochondrial process. The data suggest that MTP is oxidized via acetyl-CoA.