Table 1.

Genes Expressed Within the Human Mitochondrial Genome

Gene Name	Gene Symbol	Strand	Function	Reference
Polypeptides
Mitochondrially encoded NADH dehydrogenase 1	MT-ND1	H	Couples electron transfer from NADH to ubiquinone with transmembrane proton pumping contributing to the proton motive force used for ATP synthesis	Wirth et al. (2016)
Mitochondrially encoded NADH dehydrogenase 2	MT-ND2	H
Mitochondrially encoded NADH dehydrogenase 3	MT-ND3	H
Mitochondrially encoded NADH dehydrogenase 4	MT-ND4	H
Mitochondrially encoded NADH 4L dehydrogenase	MT-ND4L	H
Mitochondrially encoded NADH dehydrogenase 5	MT-ND5	H
Mitochondrially encoded NADH dehydrogenase 6	MT-ND6	L
Mitochondrially encoded cytochrome B	MT-CYB	H	As a component of Complex III, mediates electron transfer from ubiquinol to cytochrome c	Emmanuele et al. (2013)
Mitochondrially encoded cytochrome c oxidase I	MT-CO1	H	Cytochrome c oxidase shuttles electrons from cytochrome c to molecular oxygen to capture energy in the membrane potential by the reduction of oxygen to water	Mick et al. (2011)
Mitochondrially encoded cytochrome c oxidase II	MT-CO2	H
Mitochondrially encoded cytochrome c oxidase III	MT-CO3	H
Mitochondrially encoded ATP synthase 6	MT-ATP6	H	Produces ATP through phosphorylation of ADP by using electrochemical energy generated by proton gradient across the inner membrane of mitochondria	Neupane et al. (2019)
Mitochondrially encoded ATP synthase 8	MT-ATP8	H
Ribosomal RNA
Mitochondrially encoded 12S RNA	MT-RNR1	H	Translation of mitochondrial mRNAs together with imported ribosomal proteins	Christian and Spremulli (2012)
Mitochondrially encoded 16S RNA	MT-RNR2	H
Transfer RNA (tRNA)
Mitochondrially encoded tRNA alanine	MT-TA	L	Codon reading during mitochondrial protein translation	Scaglia and Wong (2008)
Mitochondrially encoded tRNA arginine	MT-TR	H
Mitochondrially encoded tRNA asparagine	MT-TN	L
Mitochondrially encoded tRNA aspartic acid	MT-TD	H
Mitochondrially encoded tRNA cysteine	MT-TC	L
Mitochondrially encoded tRNA glutamic acid	MT-TE	L
Mitochondrially encoded tRNA glutamine	MT-TQ	L
Mitochondrially encoded tRNA glycine	MT-TG	H
Mitochondrially encoded tRNA histidine	MT-TH	H
Mitochondrially encoded tRNA isoleucine	MT-TI	H
Mitochondrially encoded tRNA leucine 1 (UUA/G)	MT-TL1	H
Mitochondrially encoded tRNA leucine 2 (CUN)	MT-TL2	H
Mitochondrially encoded tRNA lysine	MT-TK	H
Mitochondrially encoded tRNA methionine	MT-TM	H
Mitochondrially encoded tRNA phenylalanine	MT-TF	H
Mitochondrially encoded tRNA proline	MT-TP	L
Mitochondrially encoded tRNA serine 1 (UCN)	MT-TS1	L
Mitochondrially encoded tRNA serine 2 (AGU/C)	MT-TS2	H
Mitochondrially encoded tRNA threonine	MT-TT	H
Mitochondrially encoded tRNA tryptophan	MT-TW	H
Mitochondrially encoded tRNA tyrosine	MT-TY	L
Mitochondrially encoded tRNA valine	MT-TV	H

Abbreviations: H, heavy strand; L, light strand.