Table 1.

Mitochondrial Proteins with altered function as a consequence of acetylation.

Gene ID	Gene Name	Organism1	Tissue	Site	Effect	Validation	Deacetylase	In vitro deacetylase activity with full length protein?	Ref.
ACAT1	Acetyl-CoA acetyltransferase 1	Mm	Liver	K260, K265	Inhibitory	AcK.	Sirt3	Yes	[31, 40]
AccCS2	Acetyl-CoA synthetase 2	Mm		K635	Inhibitory	AcK (PAT)	Sirt3	Yes	[13]
		Hs		K642	Inhibitory	K→Q	Sirt3	Yes	[14]
ACO2	Aconitase	Mm	Heart	n/a	Stimulatory	Chem AcK	Sirt3	No	[78]
ALDH2	Aldehyde Dehydrogenase 2	Hs		n/a	Stimulatory	n/a	Sirt3	No	[79]
ATP5E	F1F0-ATPase Subunit a	Mm	Liver	n/a	Inhibitory	No	Sirt3	No	[80]
EH/HADH	Enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase	Hs		K165, K171, K346, K584	Stimulatory	4K→Q			[22]
GLUD1	Glutamate dehydrogenase 1	Mm	Liver	n/a	Inhibitory	No	Sirt3	Yes	[50]
		Hs		n/a	Inhibitory	No	Sirt3	Yes	[67]
GOT2	Glutamate oxaloacetate transaminase	Hs		K159, K185, K404	Binding	AcK, K→R, K→Q	Sirt3	No	[81]
HMGCS2	3-Hydroxy-3-Methylglutaryl-CoA Synthase 2, Mitochondrial	Mm	Liver	K310, K447, K473	Inhibitory	K→R	Sirt3	Yes	[82]
IDH2	Isocitrate Dehydrogenase 2	Hs		n/a	Inhibitory	No	Sirt3	Yes	[67]
		Mm	Liver, Inner ear, brain	n/a	Inhibitory	No	Sirt3	Yes	[62]
		Mm	Liver	K413	Inhibitory	AcK	Sirt3	Yes	[66]
LCAD	Long chain acyl-coa dehydrogenase	Hs	Liver	K42	Inhibitory	K→R	Sirt3	Yes	[83]
		Mm	Liver	K318, K322	Inhibitory	Chemical AcK	Sirt3	Yes	[49]
MDH2	Malate Dehydrogenase 2	Hs		K185, K301, K307, K314	Stimulatory	4K→R			[22]
		Mm	Liver	K239	Inhibitory	K→Q	Sirt3	No	[33]
MRPL10	Mitochondrial Ribosomal Protein L10	Mm	Liver	n/a	Inhibitory	No	Sirt3	Yes	[84]
NDUFA9	NADH Dehydrogenase 1 a Subcomplex 9	Mm	Liver	n/a	Inhibitory	No	Sirt3	No	[85]
OGG1	8-Oxoguanine DNA glycosylase 1	Hs		n/a	Instability	No	Sirt3	No	[86]
OPA1	Optic atrophy 1	Mm	Heart	K926, K931	Inhibitory	K→Q, K→R	Sirt3	Yes	[87]
OSCP	Oligomycin sensitivity conferring protein	Hs		n/a	Inhibitory	No	Sirt3	Yes	[88]
OTC	Ornithine Transcarbamoyltransferase	Mm	Liver	K88	Inhibitory	No	Sirt3	Yes	[89]
OXCT	Succinyl CoA:3-ketoacid-CoA transferase	Mm	Brain	K451	Inhibitory	AcK	Sirt3	Yes	[40]
P450scc	P450 Cholesterol side chain cleavage monooxygenase	Hs		K148, K149	Inhibitory	K→A	Sirt3	No	[90]
PDHA	Pyruvate Dehydrogenase E1 alpha subunit	Mm	Muscle	K336	Inhibitory	K→Q, K→R	Sirt3	No	[91]
PPID	Peptidylprolyl cis-trans Isomerase D (Cyclophilin D)	Hs		K145	Binding	K→Q, K→R	Sirt3	No	[92]
		Hs		n/a	Binding	No	Sirt3	No	[93]
SDHA	Succinate Dehydrogenase Complex, Subunit A, Flavoprotein	Mm	Liver	n/a	Inhibitory	No	Sirt3	No	[94]
		Mm	Liver	n/a	Inhibitory	No	Sirt3	Yes	[95]
SKP2	F-box protein S-Phase kinase associated protein 2	Hs		K68, K71	Mislocalization	K→Q, K→R K→L	Sirt3	No	[96]
SOD2	Superoxide Dismutase 2	Mm	Liver	K122	Inhibitory	K→R	Sirt3	Yes	[63]
		Mm	Liver	K53, K89	Inhibitory	K→R	Sirt3	Yes	[65]
		Hs		K68	Inhibitory	K→Q, K→R	Sirt3	No	[64]
VLCAD	Very Long chain acyl-coa dehydrogenase	Hs	Liver	K507	Inhibitory, Mislocalization	Chemical AcK	Sirt3	Yes	[97]

¹Hs-H. sapiens, Mm-M. musculus