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. 2018 Feb 27;9(1):e00084-18. doi: 10.1128/mBio.00084-18

FIG 6 .

FIG 6 

Model of 2AA stress in the yeast mitochondrion. PLP-dependent serine dehydratases active in the yeast mitochondrion (Ilv1p and Cha1p) generate 2AA through the dehydration of serine. Unless Mmf1p is present to prevent 2AA accumulation, metabolic stress arises, ultimately causing loss of the mitochondrial genome. The precedent for 2AA damaging PLP-dependent enzymes suggests that the negative influence of 2AA on mtDNA maintenance is indirect and likely due to damage of one or more target PLP-dependent enzymes. 2KB, 2-ketobutyrate; 2AA, 2-aminoacrylate; CHA1 and ILV1 encode serine/threonine dehydratases (EC 4.3.1.19).