Table 1 List of exclusive mitochondrial proteins

1

MT000648

A kinase (PRKA) anchor protein 1

NCBI Gene ID: 8165 & UniProtKB–Q92667 (AKAP1_HUMAN) & PMID 24,412,345

2

MT000005

Acetyl-CoA carboxylase β

Gene ID: 32 & UniProtKB–O00763 (ACACB_HUMAN) & https://doi.org/10.1073/pnas.97.4.1444

3

MT000778

Acetyl-CoA acyltransferase 2

Gene ID: 10,449 & UniProtKB–P42765 (THIM_HUMAN)

4

MT000070

BCL2-antagonist/killer 1

Gene ID: 578 & UniProtKB–Q16611 (BAK_HUMAN)

5

MT000960

BCL2-binding component 3

Gene ID: 27,113 & UniProtKB–Q9BXH1 (BBC3_HUMAN)

6

MT000146

Carnitine palmitoyltransferase 1A (liver)

Gene ID: 1374 & UniProtKB–P50416 (CPT1A_HUMAN)

7

MT001561

Glycerol-3-phosphate acyltransferase 2, mitochondrial

Gene ID: 150,763 & UniProtKB–Q6NUI2 (GPAT2_HUMAN) & https://doi.org/10.1038/s41387-018-0045-x

8

MT000664

Kynurenine 3-monooxygenase (kynurenine 3-hydroxylase)

Gene ID: 8564 & UniProtKB–O15229 (KMO_HUMAN) & https://doi.org/10.1038/cddis.2016.87

9

MT000343

Monoamine oxidase B

UniProtKB P27338(AOFB_HUMAN)

PMID: 12,379,239; Gene ID: 4129

10

MT001517

Mitochondrial elongation factor 2

Gene ID: 125,170 & UniProtKB–Q96C03 (MID49_HUMAN)

11

MT000800

Metaxin 2

Gene ID: 10,651; PMID: 10,381,257 UniProtKBO75431(MTX2_HUMAN)

12

MT001642

Metaxin 3

Gene ID: 345,778; PMID: 9,045,676 UniProtKB-Q5HYI7(MTX3_HUMAN)

13

MT001315

NLR family member X1

Gene ID: 79,671 & UniProtKB–Q86UT6 (NLRX1_HUMAN)

https://doi.org/10.1038/nature06501

14

MT001589

Phospholipase D family, member 6

Gene ID: 201,164 & UniProtKB–Q8N2A8 (PLD6_HUMAN)

PMID: 25,926,691

15

MT000482

Phorbol-12-myristate-13-acetate-induced protein 1

Gene ID: 5366 & UniProtKB–Q13794 (APR_HUMAN)

16

MT001422

Mitochondria-localized glutamic acid-rich protein

UniProtKB–Q8TDB4 (HUMMR_HUMAN) &

17

MT001551

BRI3-binding protein

UniProtKB–Q8WY22 (BRI3B_HUMAN) & PMID: 17,943,721

18

MT000907

FK506-binding protein 8, 38 kDa

UniProtKB–Q14318 (FKBP8_HUMAN)

19

MT001549

FUN14 domain containing 1

UniProtKB–Q8IVP5 (FUND1_HUMAN)

20

MT000342

Monoamine oxidase A

UniProtKB–P21397 (AOFA_HUMAN)

21

MT000737

Mitofusin 2

UniProtKB–O95140 (MFN2_HUMAN) & PubMed:11,181,170

22

MT001151

Ras homolog family member T1

UniProtKB–Q8IXI2 (MIRO1_HUMAN) & PMID: 19,528,298

23

MT001448

Ras homolog family member T2

UniProtKB–Q8IXI1 (MIRO2_HUMAN)

24

MT001655

Translocase of outer mitochondrial membrane 20 homolog

(yeast)-like

UniProtKB–Q6UXN7 (TO20L_HUMAN)

25

MT001424

Ubiquitin specific peptidase 30

UniProtKB–Q70CQ3 (UBP30_HUMAN)

26

MT000626

Voltage-dependent anion channel 2

UniProtKB–P45880 (VDAC2_HUMAN)

27

MT001581

Phosphoglycerate mutase family member 5

UniProtKB–Q96HS1 (PGAM5_HUMAN) & PMID: 18,387,606

28

MT000173

Cytochrome P450, family 27, subfamily B, polypeptide 1

UniProtKB–O15528 (CP27B_HUMAN)

29

MT000554

Succinate dehydrogenase complex, subunit D, integral

membrane protein

UniProtKB–O14521 (DHSD_HUMAN)

30

MT000641

Hydroxysteroid (17-β) dehydrogenase 8

UniProtKB–Q92506 (DHB8_HUMAN)

31

MT000736

Translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae)

UniProtKB–O94826 (TOM70_HUMAN)

32

MT000779

Translocase of outer mitochondrial membrane 40 homolog

(yeast)

UniProtKB–O96008 (TOM40_HUMAN)

33

MT000918

LETM1 domain containing 1

UniProtKB–Q6P1Q0 (LTMD1_HUMAN)

34

MT001391

Translocase of outer mitochondrial membrane 40 homolog (yeast)-like

UniProtKB–Q969M1 (TM40L_HUMAN)

35

MT001597

Spermatogenesis-associated 19

UniProtKB–Q7Z5L4 (SPT19_HUMAN)

36

MT000144

Coproporphyrinogen oxidase

Gene ID: 1371 & UniProtKB–P36551 (HEM6_HUMAN)

https://doi.org/10.1016/b978-012095440-7/50031-7

37

MT000412

NADH dehydrogenase (ubiquinone) 1 β subcomplex, 7, 18 kDa

Gene ID: 4713 & UniProtKB–P17568 (NDUB7_HUMAN)

38

MT000751

Peptidylprolyl isomerase F

Gene ID: 10,105 & UniProtKB–P30405 (PPIF_HUMAN)

PMID: 10,406,942

39

MT000581

Steroidogenic acute regulatory protein

Gene ID: 6770 & UniProtKB–P49675 (STAR_HUMAN)

https://doi.org/10.1073/pnas.93.24.13731

40

MT001408

Apoptogenic 1, mitochondrial IMM

UniProtKB–Q96IL0 (COA8_HUMAN)

41

MT001533

Coiled-coil-helix-coiled-coil-helix domain containing 4 Mito inter memb. space

UniProtKB–Q8N4Q1 (MIA40_HUMAN)

42

MT000972

HtrA serine peptidase 2 MIM

UniProtKB–O43464 (HTRA2_HUMAN)

43

MT000773

Mitochondrial calcium uptake 1 MIM

UniProtKB–Q9BPX6 (MICU1_HUMAN)

44

MT001600

Mitochondrial calcium uptake 2 MIM

UniProtKB–Q8IYU8 (MICU2_HUMAN)

45

MT000418

NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75 kDa

(NADH-coenzyme Q reductase) MIM

UniProtKB–P28331 (NDUS1_HUMAN)

46

MT000448

Optic atrophy 1 (autosomal dominant) MIM

UniProtKB–O60313 (OPA1_HUMAN)

47

MT000947

Family with sequence similarity 162, member A

UniProtKB–Q96A26 (F162A_HUMAN)

48

MT001404

Ubiquinol-cytochrome c reductase complex assembly factor 2

UniProtKB–Q9BRT2 (UQCC2_HUMAN)

49

MT000002

ATP-binding cassette, subfamily B (MDR/TAP), member 7

UniProtKB–O75027 (ABCB7_HUMAN)

50

MT000843

ATP-binding cassette, subfamily B (MDR/TAP), member 8

UniProtKB–Q9NUT2 (MITOS_HUMAN)

51

MT001559

Apoptosis-inducing factor, mitochondrion-associated, 3

UniProtKB–Q96NN9 (AIFM3_HUMAN)

52

MT000526

Aldehyde dehydrogenase 18 family, member A1

UniProtKB–P54886 (P5CS_HUMAN)

53

MT001548

Apolipoprotein O-like

UniProtKB–Q6UXV4 (MIC27_HUMAN)

54

MT001145

ATPase family, AAA domain containing 3A

UniProtKB–Q9NVI7 (ATD3A_HUMAN)

55

MT001384

ATPase family, AAA domain containing 3B

UniProtKB–Q5T9A4 (ATD3B_HUMAN)

56

MT000081

3-Hydroxybutyrate dehydrogenase, type 1

UniProtKB–Q02338 (BDH_HUMAN)

57

MT001121

Coiled-coil domain containing 109B

UniProtKB–Q9NWR8 (MCUB_HUMAN)

58

MT001405

Coiled-coil-helix-coiled-coil-helix domain containing 6

UniProtKB–Q9BRQ6 (MIC25_HUMAN)

58

MT001158

Choline dehydrogenase

UniProtKB–Q8NE62 (CHDH_HUMAN)

60

MT001679

Creatine kinase, mitochondrial 1A

UniProtKB–P12532 (KCRU_HUMAN)

61

MT000120

Creatine kinase, mitochondrial 2 (sarcomeric)

UniProtKB–P17540 (KCRS_HUMAN)

62

MT000966

Coenzyme Q2 4-hydroxybenzoate polyprenyltransferase

UniProtKB–Q96H96 (COQ2_HUMAN)

63

MT001035

Coenzyme Q4

UniProtKB–Q9Y3A0 (COQ4_HUMAN)

64

MT001206

Coenzyme Q9

UniProtKB–O75208 (COQ9_HUMAN)

65

MT000141

COX10 heme A: farnesyltransferase cytochrome c oxidase

assembly factor

UniProtKB–Q12887 (COX10_HUMAN)

66

MT000142

COX11 cytochrome c oxidase copper chaperone

UniProtKB–Q9Y6N1 (COX11_HUMAN)

67

MT000753

TNF receptor-associated protein 1

UniProtKB–Q12931 (TRAP1_HUMAN) (PMID: 23,564,345)

68

MT001419

Cytochrome c oxidase subunit IV isoform 2 (lung)

UniProtKB–Q96KJ9 (COX42_HUMAN)

69

MT000705

Cytochrome c oxidase subunit Va

UniProtKB–P20674(COX5A_HUMAN)

70

MT000130

Cytochrome c oxidase subunit Vb

UniProtKB–P10606 (COX5B_HUMAN)

71

MT000131

Cytochrome c oxidase subunit VIa polypeptide 1

UniProtKB–P12074 (CX6A1_HUMAN)

72

MT000132

Cytochrome c oxidase subunit VIa polypeptide 2

UniProtKB–Q02221 (CX6A2_HUMAN)

73

MT000133

Cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)

UniProtKB–P14854 (CX6B1_HUMAN)

74

MT000134

Cytochrome c oxidase subunit VIc

UniProtKB–P09669 (COX6C_HUMAN)

75

MT000135

Cytochrome c oxidase subunit VIIa polypeptide 1 (muscle)

UniProtKB–P24310 (CX7A1_HUMAN)

76

MT000805

YME1-like 1 ATPase

UniProtKB-Q96TA2(YMEL1_HUMAN)

PMID: 10,843,804; PMID: 22,262,461

77

MT000699

Cytochrome c oxidase subunit VIIa polypeptide 2-like

UniProtKB–O14548(COX7R_HUMAN)

78

MT000138

Cytochrome c oxidase subunit VIIb

UniProtKB–P24311 (COX7B_HUMAN)

79

MT001093

Cardiolipin synthase 1

UniProtKB–Q9UJA2(CRLS1_HUMAN)

80

MT000162

Cytochrome c-1

UniProtKB–P08574 (CY1_HUMAN)

81

MT000958

Ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5 kDa

UniProtKB–O14949 (QCR8_HUMAN)

82

MT000182

Dihydroorotate dehydrogenase (quinone)

UniProtKB–Q02127 (PYRD_HUMAN)

83

MT001172

DnaJ (Hsp40) homolog, subfamily C, member 11

UniProtKB–Q9NVH1(DJC11_HUMAN)

84

MT000990

DnaJ (Hsp40) homolog, subfamily C, member 15

UniProtKB–Q9Y5T4 (DJC15_HUMAN)

85

MT000622

Ubiquinol-cytochrome c reductase hinge protein

UniProtKB–P07919 (QCR6_HUMAN)

86

MT000177

Enoyl-CoA δ isomerase 1

UniProtKB–P42126 (ECI1_HUMAN)

87

MT000223

Ferredoxin reductase

UniProtKB–P22570 (ADRO_HUMAN)

88

MT000224

Ferrochelatase

UniProtKB–P22830 (HEMH_HUMAN)

89

MT000240

Glutaryl-CoA dehydrogenase

UniProtKB–Q92947 (GCDH_HUMAN)

90

MT000957

Growth hormone inducible transmembrane protein

UniProtKB-Q9H3K2(GHITM_HUMAN)

91

MT000262

Glycerol-3-phosphate dehydrogenase 2 (mitochondrial)

UniProtKB–P43304 (GPDM_HUMAN)

92

MT000279

Hydroxyacyl-CoA dehydrogenase

UniProtKB–Q16836 (HCDH_HUMAN)

93

MT000277

Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), α subunit

UniProtKB–P40939 (ECHA_HUMAN)

94

MT000929

HIG1 hypoxia inducible domain family, member 1A

UniProtKB–Q9Y241(HIG1A_HUMAN)

95

MT001582

HIG1 hypoxia inducible domain family, member 2A

UniProtKB–Q9BW72(HIG2A_HUMAN)

96

MT000275

Hydroxysteroid (17-β) dehydrogenase 10

UniProtKB–Q99714 (HCD2_HUMAN)

PMID: 12,917,011 PMID: 18,984,158

97

MT000312

Isocitrate dehydrogenase 2 (NADP +), mitochondrial

UniProtKB–P48735 (IDHP_HUMAN)

98

MT000827

Inner membrane protein, mitochondrial

UniProtKB–Q16891 (MIC60_HUMAN)

99

MT001584

Lactate dehydrogenase D

UniProtKB–Q86WU2(LDHD_HUMAN)

100

MT001068

Mitochondrial pyruvate carrier 1

UniProtKB–Q9Y5U8 (MPC1_HUMAN)

101

MT000917

Mitochondrial pyruvate carrier 2

UniProtKB–O95563 (MPC2_HUMAN)

102

MT000366

MpV17 mitochondrial inner membrane protein

UniProtKB–P39210 (MPV17_HUMAN)

PMID: 16,582,907 PMID: 16,582,910

103

MT001218

MRS2 magnesium transporter

UniProtKB–Q9HD23 (MRS2_HUMAN)

104

MT001675

Methylenetetrahydrofolate dehydrogenase (NADP + dependent) 2-like

UniProtKB–Q9H903(MTD2L_HUMAN)

105

MT000395

NADH dehydrogenase (ubiquinone) 1 α subcomplex, 1, 7.5 kDa

UniProtKB–O15239(NDUA1_HUMAN)

106

MT001668

Solute carrier family 25, member 53

UniProtKB–Q5H9E4 (S2553_HUMAN)

107

MT001701

NDUFC2-KCTD14 readthrough

UniProtKB E9PQ53(NDUCR_HUMAN)

108

MT000421

NADH dehydrogenase (ubiquinone) flavoprotein 1, 51 kDa

UniProtKB–P49821(NDUV1_HUMAN)

109

MT000434

NME/NM23 nucleoside diphosphate kinase 4

UniProtKB–O00746 (NDKM_HUMAN)

110

MT000894

Nicotinamide nucleotide transhydrogenase

UniProtKB–Q13423 (NNTM_HUMAN)

111

MT001400

Nitric oxide-associated 1

UniProtKB–Q8NC60 (NOA1_HUMAN)

112

MT001491

OMA1 zinc metallopeptidase

UniProtKB–Q96E52 (OMA1_HUMAN)

113

MT000451

Oxidase (cytochrome c) assembly 1-like

UniProtKB–Q15070(OXA1L_HUMAN)

114

MT000457

Pyruvate carboxylase

UniProtKB–P11498 (PYC_HUMAN)

115

MT000468

Pyruvate dehydrogenase kinase, isozyme 4

UniProtKB–Q16654 (PDK4_HUMAN)

116

MT000714

Phosphatidylglycerophosphate synthase 1

UniProtKB–Q32NB8(PGPS1_HUMAN)

117

MT000873

Peptidase (mitochondrial processing) α

UniProtKB–Q10713 (MPPA_HUMAN); PMID: 25,808,372

118

MT000715

Peptidase (mitochondrial processing) β

UniProtKB–O75439 (MPPB_HUMAN); PMID: 22,354,088

119

MT000484

Polymerase (DNA directed), γ

UniProtKB–P54098 (DPOG1_HUMAN); PMID: 18,063,578

120

MT000964

PRELI domain containing 1

UniProtKB–Q9Y255 (PRLD1_HUMAN)

121

MT000511

Proline dehydrogenase (oxidase) 1

UniProtKB–O43272 (PROD_HUMAN)

122

MT001488

Protein tyrosine phosphatase, mitochondrial 1

UniProtKB–Q8WUK0 (PTPM1_HUMAN)

123

MT000621

Ubiquinol-cytochrome c reductase, Rieske iron sulfur polypeptide 1

UniProtKB–P47985 (UCRI_HUMAN)

124

MT000618

Ubiquinol-cytochrome c reductase-binding protein

UniProtKB–P14927 (QCR7_HUMAN)

125

MT000549

SCO1 cytochrome c oxidase assembly protein

UniProtKB–O75880 (SCO1_HUMAN); PMID: 15,229,189

126

MT000740

SCO2 cytochrome c oxidase assembly protein

UniProtKB–O43819 (SCO2_HUMAN); PMID: 15,229,189

127

MT000551

Succinate dehydrogenase complex, subunit A, flavoprotein (Fp)

UniProtKB–P31040 (SDHA_HUMAN)

128

MT000552

Succinate dehydrogenase complex, subunit B, iron sulfur (Ip)

UniProtKB–P21912 (SDHB_HUMAN)

129

MT001478

sideroflexin 1

UniProtKB–Q9H9B4 (SFXN1_HUMAN); PMID: 30,442,778

130

MT000564

Solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1

UniProtKB–P53007 (TXTP_HUMAN)

131

MT000035

Solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 5

UniProtKB–P05141 (ADT2_HUMAN)

132

MT001465

Solute carrier family 25, member 51

UniProtKB–Q9H1U9 (S2551_HUMAN)

133

MT000587

surfeit 1

UniProtKB–Q15526 (SURF1_HUMAN)

134

MT001534

TAM41, mitochondrial translocator assembly and maintenance

protein, homolog (S. cerevisiae)

UniProtKB-Q96BW9(TAM41_HUMAN)

135

MT001393

Transmembrane protein 126A

UniProtKB–Q9H061(T126A_HUMAN); PMID: 19,327,736; PMID: 23,500,070

136

MT001109

Transmembrane protein 70

UniProtKBQ9BUB7(TMM70_HUMAN);

PMID: 20,937,241

137

MT000605

Thiosulfate sulfurtransferase (rhodanese)

UniProtKB–Q16762 (THTR_HUMAN)

138

MT001099

Tetratricopeptide repeat domain 19

UniProtKB–Q6DKK2(TTC19_HUMAN)

PMID: 21,278,747

139

MT000992

Ubiquinol-cytochrome c reductase, complex III subunit X

UniProtKB–Q9UDW1(QCR9_HUMAN)

140

MT000825

Ubiquinol-cytochrome c reductase, complex III subunit XI

UniProtKB–O14957 (QCR10_HUMAN)

141

MT000053

ATP synthase, H + transporting, mitochondrial F1 complex, δ subunit

UniProtKB–P30049 (ATPD_HUMAN),

142

MT000054

ATP synthase, H + transporting, mitochondrial F1 complex, ε subunit

UniProtKB–P56381 (ATP5E_HUMAN),

143

MT000055

ATP synthase, H + transporting, mitochondrial Fo complex, subunit B1

UniProtKB–P24539 (AT5F1_HUMAN),

144

MT000056

ATP synthase, H + transporting, mitochondrial Fo complex, subunit C1 (subunit 9)

UniProtKB–P05496 (AT5G1_HUMAN),

145

MT000058

ATP synthase, H + transporting, mitochondrial Fo complex, subunit C3 (subunit 9)

UniProtKB–P48201 (AT5G3_HUMAN),

146

MT000098

Solute carrier family 25 (carnitine/acylcarnitine translocase), member 20

UniProtKB–O43772 (MCAT_HUMAN),

147

MT000156

Solute carrier family 25 (mitochondrial carrier; dicarboxylate transporter), member 10

UniProtKB–Q9UBX3 (DIC_HUMAN),

148

MT000171

Cytochrome P450, family 24, subfamily A, polypeptide 1

UniProtKB–Q07973 (CP24A_HUMAN)

149

MT000333

Leucine zipper-EF-hand-containing transmembrane protein 1

UniProtKB–O95202(LETM1_HUMAN)

150

MT000407

NADH dehydrogenase (ubiquinone) 1 β subcomplex, 2, 8 kDa

UniProtKB–O95178(NDUB2_HUMAN)

151

MT000413

NADH dehydrogenase (ubiquinone) 1 β subcomplex, 8, 19 kDa

UniProtKB–O95169(NDUB8_HUMAN)

152

MT000414

NADH dehydrogenase (ubiquinone) 1 β subcomplex, 9, 22 kDa

UniProtKB Q9Y6M9(NDUB9_HUMAN)

153

MT000416

NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6 kDa

UniProtKB–O43677(NDUC1_HUMAN)

154

MT000419

NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49 kDa

(NADH-coenzyme Q reductase)

UniProtKB–O75306(NDUS2_HUMAN)

155

MT000420

NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30 kDa

(NADH-coenzyme Q reductase)

UniProtKB–O75489(NDUS3_HUMAN)

156

MT000426

NADH dehydrogenase (ubiquinone) flavoprotein 2, 24 kDa

UniProtKB–P19404(NDUV2_HUMAN)

157

MT000427

NADH dehydrogenase (ubiquinone) flavoprotein 3, 10 kDa

UniProtKB–P56181(NDUV3_HUMAN)

158

MT000474

Solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3

UniProtKB–Q00325 (MPCP_HUMAN)

159

MT000570

Superoxide dismutase 2, mitochondrial

UniProtKB–P04179 (SODM_HUMAN)

160

MT000620

Ubiquinol-cytochrome c reductase core protein II

UniProtKB–P22695 (QCR2_HUMAN)

161

MT000645

Solute carrier family 25 (mitochondrial carrier), member 16

UniProtKB–P16260 (GDC_HUMAN)

162

MT000654

Solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11

UniProtKB–Q02978 (M2OM_HUMAN)

163

MT001641

Cytochrome c oxidase subunit VIIIC

UniProtKB–Q7Z4L0(COX8C_HUMAN)

164

MT000948

Translocase of inner mitochondrial membrane 10 homolog B

UniProtKB–Q9Y5J6 (T10B_HUMAN)

165

MT001052

Solute carrier family 25 (mitochondrial iron transporter), member 37

UniProtKB Q9NYZ2(MFRN1_HUMAN)

166

MT001059

Mitochondrial fission process 1

UniProtKB Q9UDX5(MTFP1_HUMAN)

167

MT001088

NADH dehydrogenase (ubiquinone) 1 β subcomplex, 11, 17.3 kDa

UniProtKB Q9NX14(NDUBB_HUMAN)

168

MT001233

Sulfide quinone reductase-like

UniProtKB–Q9Y6N5 (SQOR_HUMAN)

169

MT001306

NADH dehydrogenase (ubiquinone) complex I, assembly factor 5

UniProtKB Q5TEU4(NDUF5_HUMAN)

170

MT001324

Solute carrier family 25 (mitochondrial carrier: glutamate), member 22

UniProtKB–Q9H936 (GHC1_HUMAN)

171

MT001336

L-2-hydroxyglutarate dehydrogenase

UniProtKB–Q9H9P8(L2HDH_HUMAN)

172

MT001474

Coenzyme Q10 homolog A

UniProtKB–Q96MF6(CQ10A_HUMAN)

173

MT001479

Sideroflexin 5

UniProtKB–Q8TD22(SFXN5_HUMAN)

174

MT001507

Sideroflexin 4

UniProtKB–Q6P4A7(SFXN4_HUMAN)

175

MT000809

Caseinolytic mitochondrial matrix peptidase chaperone subunit

UniProtKB–O76031 (CLPX_HUMAN)

176

MT000713

Solute carrier family 25, member 27

UniProtKB–O95847 (UCP4_HUMAN)

177

MT000059

ATP synthase, H + transporting, mitochondrial Fo complex, subunit E

UniProtKB–P56385 (ATP5I_HUMAN)

178

MT000060

ATP synthase, H + transporting, mitochondrial Fo complex, subunit F6

UniProtKB–P18859 (ATP5J_HUMAN)

179

MT000064

ATP synthase, H + transporting, mitochondrial F1 complex, O subunit

UniProtKB–P48047 (ATPO_HUMAN)

180

MT000143

Cytochrome c oxidase assembly homolog 15 (yeast)

UniProtKB Q7KZN9(COX15_HUMAN)

181

MT000396

NADH dehydrogenase (ubiquinone) 1 α subcomplex, 2, 8 kDa

UniProtKB–O43678(NDUA2_HUMAN)

182

MT000404

NADH dehydrogenase (ubiquinone) 1 α subcomplex, 10, 42 kDa

UniProtKB–O95299(NDUAA_HUMAN)

183

MT000415

NADH dehydrogenase (ubiquinone) 1 β subcomplex, 10, 22 kDa

UniProtKB–O96000(NDUBA_HUMAN)

184

MT000560

Solute carrier family 3 (amino acid transporter heavy chain), member 1

UniProtKB–Q07837 (SLC31_HUMAN)

185

MT000666

Solute carrier family 25 (aspartate/glutamate carrier), member 12

UniProtKB–O75746 (CMC1_HUMAN)

186

MT000720

ATP synthase, H + transporting, mitochondrial Fo complex, subunit F2

UniProtKB–P56134 (ATPK_HUMAN)

187

MT000738

Endo/exonuclease (5'-3'), endonuclease G-like

UniProtKB–Q9Y2C4 (EXOG_HUMAN)

188

MT000783

ATP synthase, H + transporting, mitochondrial Fo complex, subunit d

UniProtKB–O75947 (ATP5H_HUMAN)

189

MT000853

Tumor suppressor candidate 2

UniProtKB–O75896 (TUSC2_HUMAN)

190

MT000889

ATP-binding cassette, subfamily B (MDR/TAP), member 10

UniProtKBQ9NRK6 (ABCBA_HUMAN)

191

MT000909

Mitochondrial carrier 1

UniProtKB Q9NZJ7 (MTCH1_HUMAN)

192

MT000910

Mitochondrial carrier 2

UniProtKB Q9Y6C9 (MTCH2_HUMAN)

193

MT001672

Mitochondrial inner membrane organizing system 1

UniProtKB–Q5TGZ0 (MIC10_HUMAN)

194

MT000997

Solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 24

UniProtKB-Q6NUK1(SCMC1_HUMAN)

195

MT001024

NADH dehydrogenase (ubiquinone) 1 α subcomplex, 13

UniProtKB Q9P0J0 (NDUAD_HUMAN)

196

MT001030

NADH dehydrogenase (ubiquinone) complex I, assembly factor 1

UniProtKB–Q9Y375 (CIA30_HUMAN)

197

MT001063

Solute carrier family 25, member 39

UniProtKB–Q9BZJ4 (S2539_HUMAN)

198

MT001111

Solute carrier family 25, member 38

UniProtKB–Q96DW6 (S2538_HUMAN)

199

MT001143

Solute carrier family 25 (pyrimidine nucleotide carrier), member 36

UniProtKB–Q96CQ1 (S2536_HUMAN)

200

MT001187

Solute carrier family 25, member 40

UniProtKB–Q8TBP6 (S2540_HUMAN)

201

MT001242

GUF1 GTPase homolog (S. cerevisiae)

UniProtKB–Q8N442 (GUF1_HUMAN)

202

MT001244

Mitochondrial ribosomal protein L17

UniProtKB–Q9NRX2 (RM17_HUMAN)

203

MT001249

Mitochondrial calcium uniporter regulator 1

UniProtKBQ96AQ8 (MCUR1_HUMAN)

204

MT001305

Solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 23

UniProtKB Q9BV35 (SCMC3_HUMAN)

205

MT001339

Solute carrier family 8 (sodium/lithium/calcium exchanger), member B1

UniProtKB–Q6J4K2 (NCLX_HUMAN)

206

MT001346

Coenzyme Q10 homolog B (S. cerevisiae)

UniProtKB Q9H8M1 (CQ10B_HUMAN)

207

MT001352

EF-hand domain family, member D1

UniProtKB Q9BUP0 (EFHD1_HUMAN)

208

MT001361

Solute carrier family 25 (mitochondrial folate carrier), member 32

UniProtKB–Q9H2D1 (MFTC_HUMAN)

209

MT001381

Solute carrier family 25 (glutamate carrier), member 18

UniProtKB–Q9H1K4 (GHC2_HUMAN)

210

MT001386

Solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 2

UniProtKB–Q9BXI2 (ORNT2_HUMAN)

211

MT001388

Spinster homolog 1 (Drosophila)

UniProtKB–Q9H2V7 (SPNS1_HUMAN)

212

MT001402

Solute carrier family 25 (pyrimidine nucleotide carrier), member 33

UniProtKB–Q9BSK2 (S2533_HUMAN)

213

MT001446

Solute carrier family 25 (mitochondrial oxoadipate carrier), member 21

UniProtKB–Q9BQT8 (ODC_HUMAN)

214

MT001453

Mitochondrial calcium uniporter

UniProtKB–Q8NE86 (MCU_HUMAN)

215

MT001458

Solute carrier family 25, member 46

UniProtKB–Q96AG3 (S2546_HUMAN)

216

MT001463

Single-pass membrane protein with aspartate-rich tail 1

UniProtKB–Q9H4I9 (EMRE_HUMAN)

217

MT001487

Solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 25

UniProtKBQ6KCM7 (SCMC2_HUMAN)

218

MT001492

Solute carrier family 25 (S-adenosylmethionine carrier), member 26

UniProtKB Q70HW3 (SAMC_HUMAN)

219

MT001511

Solute carrier family 25 (mitochondrial

carnitine/acylcarnitine carrier), member 29

UniProtKBQ8N8R3 (MCATL_HUMAN)

220

MT001541

GrpE-like 2, mitochondrial (E. coli)

UniProtKB Q8TAA5 (GRPE2_HUMAN)

221

MT001546

Leucine zipper-EF-hand containing transmembrane protein 2

UniProtKB Q2VYF4 (LETM2_HUMAN)

222

MT001558

Solute carrier family 25, member 52

UniProtKB–Q3SY17 (S2552_HUMAN)

223

MT001565

Solute carrier family 25, member 48

UniProtKB–Q6ZT89 (S2548_HUMAN)

224

MT001579

Cytochrome c oxidase subunit VIIb2

UniProtKB–Q8TF08 (CX7B2_HUMAN)

225

MT001594

Solute carrier family 25, member 43

UniProtKB–Q8WUT9 (S2543_HUMAN)

226

MT001605

Solute carrier family 25, member 30

UniProtKB Q5SVS4 (KMCP1_HUMAN)

227

MT001614

ATP synthase, H + transporting, mitochondrial Fo complex, subunit G2

UniProtKB–Q7Z4Y8 (AT5L2_HUMAN)

228

MT001617

Solute carrier family 25, member 45

UniProtKB–Q8N413 (S2545_HUMAN)

229

MT001621

Solute carrier family 25, member 47

UniProtKB–Q6Q0C1 (S2547_HUMAN)

230

MT001627

Solute carrier family 25, member 41

UniProtKB–Q8N5S1 (S2541_HUMAN)

231

MT001628

Solute carrier family 25, member 42

UniProtKB–Q86VD7 (S2542_HUMAN)

232

MT001630

Solute carrier family 25, member 34

UniProtKB–Q6PIV7 (S2534_HUMAN)

233

MT001643

Mitochondrial pyruvate carrier 1-like

UniProtKB P0DKB6 (MPC1L_HUMAN)

234

MT001648

NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20 kDa

(NADH-coenzyme Q reductase)

UniProtKB–O75251 (NDUS7_HUMAN)

235

MT001661

Solute carrier family 25, member 35

UniProtKB–Q3KQZ1 (S2535_HUMAN)

236

MT000140

Cytochrome c oxidase subunit VIIIA (ubiquitous)

UniProtKB–P10176 (COX8A_HUMAN)

237

MT000169

Cytochrome P450, family 11, subfamily B, polypeptide 2

UniProtKB–P19099 (C11B2_HUMAN)

238

MT000760

Coenzyme Q7 homolog, ubiquinone (yeast)

UniProtKB–Q99807 (COQ7_HUMAN)

239

MT000755

Aminoadipate-semialdehyde synthase

UniProtKB–Q9UDR5 (AASS_HUMAN)

240

MT000001

4-Aminobutyrate aminotransferase

UniProtKB–P80404 (GABT_HUMAN), PMID: 15,528,998

241

MT000954

Acyl-CoA dehydrogenase family, member 8

UniProtKB–Q9UKU7 (ACAD8_HUMAN), PMID: 11,013,134; PMID: 12,359,132

242

MT000006

Acyl-CoA dehydrogenase, long chain

UniProtKB–P28330 (ACADL_HUMAN)

243

MT000007

Acyl-CoA dehydrogenase, C-4 to C-12 straight chain

UniProtKB–P11310 (ACADM_HUMAN)

244

MT000008

Acyl-CoA dehydrogenase, C-2 to C-3 short chain

UniProtKB–P16219 (ACADS_HUMAN)

245

MT000009

Acyl-CoA dehydrogenase, short/branched chain

UniProtKB–P45954 (ACDSB_HUMAN)

246

MT000010

Acyl-CoA dehydrogenase, very long chain

UniProtKB–P49748 (ACADV_HUMAN)

247

MT000011

Acetyl-CoA acetyltransferase 1

UniProtKB–P24752 (THIL_HUMAN), PMID: 1,979,337

248

MT000014

Aconitase 2, mitochondrial

UniProtKB–Q99798 (ACON_HUMAN)

249

MT001497

Acyl-CoA synthetase medium-chain family member 1

UniProtKB–Q08AH1 (ACSM1_HUMAN)

250

MT001644

Acyl-CoA synthetase medium-chain family member 2B

UniProtKB–Q68CK6 (ACS2B_HUMAN), PMID: 12,616,642; PMID: 10,434,065; PMID: 19,634,011

251

MT000546

Acyl-CoA synthetase medium-chain family member 3

UniProtKB–Q53FZ2 (ACSM3_HUMAN)

252

MT001411

Acyl-CoA synthetase short-chain family member 1

UniProtKB–Q9NUB1 (ACS2L_HUMAN), PMID: 16,788,062

253

MT001545

alcohol dehydrogenase, iron containing, 1

UniProtKB–Q8IWW8 (HOT_HUMAN)

254

MT001271

Alanine–glyoxylate aminotransferase 2

UniProtKB–Q9BYV1 (AGT2_HUMAN), PMID: 20,018,850; PMID: 24,586,340

255

MT001005

Adenylate kinase 3

UniProtKB–Q9UIJ7 (KAD3_HUMAN), PMID: 11,485,571

256

MT000020

Adenylate kinase 4

UniProtKB–P27144 (KAD4_HUMAN), PMID: 11,485,571; PMID: 19,766,732; PMID: 26,980,435

257

MT000022

5′-Aminolevulinate synthase 1

UniProtKB–P13196 (HEM1_HUMAN)

258

MT000023

5′-Aminolevulinate synthase 2

UniProtKB–P22557 (HEM0_HUMAN), PMID: 14,643,893

259

MT000026

Aldehyde dehydrogenase 2 family (mitochondrial)

UniProtKB–P05091 (ALDH2_HUMAN)

260

MT001397

alkB, alkylation repair homolog 7 (E. coli)

UniProtKB–Q9BT30 (ALKB7_HUMAN), PMID: 23,666,923

261

MT000041

Arginase 2

UniProtKB–P78540 (ARGI2_HUMAN), PMID: 8,898,077

262

MT000066

AU RNA-binding protein/enoyl-CoA hydratase

UniProtKB–Q13825 (AUHM_HUMAN)

263

MT000074

Branched chain keto acid dehydrogenase E1, α polypeptide

UniProtKB–P12694 (ODBA_HUMAN)

264

MT000075

Branched chain keto acid dehydrogenase E1, β polypeptide

UniProtKB–P21953 (ODBB_HUMAN)

265

MT000097

Carbonic anhydrase VA, mitochondrial

UniProtKB–P35218 (CAH5A_HUMAN)

266

MT000849

Carbonic anhydrase VB, mitochondrial

UniProtKB–Q9Y2D0(CAH5B_HUMAN)

267

MT001428

Carbonyl reductase 4

UniProtKB–Q8N4T8 (CBR4_HUMAN), PMID: 19,000,905 PMID: 19,571,038

268

MT000649

Caseinolytic mitochondrial matrix peptidase proteolytic subunit

UniProtKB–Q16740 (CLPP_HUMAN), PMID: 10,525,407 PMID: 22,354,088

269

MT000154

citrate synthase

UniProtKB–O75390 (CISY_HUMAN), PMID: 9,543,345

270

MT000167

Cytochrome P450, family 11, subfamily A, polypeptide 1

UniProtKB–P05108 (CP11A_HUMAN)

271

MT000172

Cytochrome P450, family 27, subfamily A, polypeptide 1

UniProtKB–Q02318 (CP27A_HUMAN)

The Human protein ATLAS

272

MT001686

D-2-hydroxyglutarate dehydrogenase

UniProtKB–Q8N465 (D2HDH_HUMAN), PMID: 15,070,399

The Human protein ATLAS

273

MT000639

Death-associated protein 3

UniProtKB–P51398 (RT29_HUMAN), PMID: 11,162,496; PMID: 20,563,667

274

MT001138

Aspartyl-tRNA synthetase 2, mitochondrial

UniProtKB–Q6PI48 (SYDM_HUMAN), PMID: 15,779,907

275

MT000176

Dihydrolipoamide branched chain transacylase E2

UniProtKB–P11182 (ODB2_HUMAN)

276

MT000181

Deoxyguanosine kinase

UniProtKB–Q16854 (DGUOK_HUMAN)

277

MT001587

Dihydrofolate reductase-like 1

UniProtKB–Q86XF0 (DYR2_HUMAN), PMID: 21,876,188

278

MT000185

Dihydrolipoamide S-acetyltransferase

UniProtKB–P10515 (ODP2_HUMAN)

279

MT000998

Dimethylglycine dehydrogenase

UniProtKB–Q9UI17 (M2GD_HUMAN)

280

MT000199

Enoyl-CoA hydratase, short chain, 1, mitochondrial

UniProtKB–P30084 (ECHM_HUMAN)

281

MT000945

Era-like 12S mitochondrial rRNA chaperone 1

UniProtKB–O75616 (ERAL1_HUMAN)

282

MT000212

Electron-transfer-flavoprotein, α polypeptide

UniProtKB–P13804 (ETFA_HUMAN)

283

MT000213

Electron-transfer-flavoprotein, β polypeptide

UniProtKB–P38117 (ETFB_HUMAN), PMID: 8,504,797; PMID: 25,023,281

284

MT000214

Electron-transferring-flavoprotein dehydrogenase

UniProtKB–Q16134 (ETFD_HUMAN)

285

MT000222

Ferredoxin 1

UniProtKB–P10109 (ADX_HUMAN), PMID: 20,547,883

286

MT000963

Glutaminase 2 (liver, mitochondrial)

UniProtKB–Q9UI32 (GLSL_HUMAN), PMID: 20,378,837

287

MT000255

Glutamate dehydrogenase 1

UniProtKB–P00367 (DHE3_HUMAN)

288

MT000765

Glycine-N-acyltransferase

UniProtKB–Q6IB77 (GLYAT_HUMAN), PMID: 22,475,485

289

MT001421

Glutamic pyruvate transaminase (alanine aminotransferase) 2

UniProtKB–Q8TD30 (ALAT2_HUMAN)

290

MT000887

Histidyl-tRNA synthetase 2, mitochondrial

UniProtKB–P49590 (SYHM_HUMAN), PMID: 21,464,306

291

MT000839

3-Hydroxyisobutyrate dehydrogenase

UniProtKB–P31937 (3HIDH_HUMAN)

292

MT000943

3-Hydroxyisobutyryl-CoA hydrolase

UniProtKB–Q6NVY1 (HIBCH_HUMAN)

293

MT000292

3-Hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)

UniProtKB–P54868 (HMCS2_HUMAN)

294

MT000306

Heat shock 60 kDa protein 1 (chaperonin)

UniProtKB–P10809 (CH60_HUMAN)

295

MT001171

Isoleucyl-tRNA synthetase 2, mitochondrial

UniProtKB–Q9NSE4 (SYIM_HUMAN)

296

MT000309

Immature colon carcinoma transcript 1

UniProtKB–Q14197 (ICT1_HUMAN), PMID: 20,186,120 PMID: 23,908,630

297

MT000313

Isocitrate dehydrogenase 3 (NAD +) α

UniProtKB–P50213 (IDH3A_HUMAN)

298

MT000314

Isocitrate dehydrogenase 3 (NAD +) β

UniProtKB–O43837 (IDH3B_HUMAN)

299

MT000324

Isovaleryl-CoA dehydrogenase

UniProtKB–P26440 (IVD_HUMAN)

300

MT000880

Leucyl-tRNA synthetase 2, mitochondrial

UniProtKB–Q15031 (SYLM_HUMAN)

301

MT001473

Methionyl-tRNA synthetase 2, mitochondrial

UniProtKB–Q96GW9(SYMM_HUMAN)

302

MT001197

Methylcrotonoyl-CoA carboxylase 1 (α)

UniProtKB–Q96RQ3 (MCCA_HUMAN), PMID: 16,023,992

303

MT001254

Methylcrotonoyl-CoA carboxylase 2 (β)

UniProtKB–Q9HCC0 (MCCB_HUMAN), PMID: 11,170,888; PMID: 16,023,992

304

MT001418

Methylmalonyl CoA epimerase

UniProtKB–Q96PE7 (MCEE_HUMAN)

305

MT000350

Malate dehydrogenase 2, NAD (mitochondrial)

UniProtKB–P40926 (MDHM_HUMAN)

306

MT001472

Mitochondrial genome maintenance exonuclease 1

UniProtKB–Q9BQP7 (MGME1_HUMAN), PMID: 23,313,956 PMID: 23,358,826

307

MT001575

Methylmalonic aciduria (cobalamin deficiency) cblA type

UniProtKB–Q8IVH4 (MMAA_HUMAN)

308

MT001634

Methylmalonic aciduria (cobalamin deficiency) cblB type

UniProtKB–Q96EY8 (MMAB_HUMAN)

309

MT001515

Mitochondrial ribosomal protein L10

UniProtKB–Q7Z7H8 (RM10_HUMAN), PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

310

MT001286

Mitochondrial ribosomal protein L11

UniProtKB–Q9Y3B7 (RM11_HUMAN), PMID: 11,279,069 PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

311

MT000541

Mitochondrial ribosomal protein L12

UniProtKB–P52815 (RM12_HUMAN), PMID: 8,626,705

312

MT000983

Mitochondrial ribosomal protein L13

UniProtKB–Q9BYD1 (RM13_HUMAN), PMID: 25,278,503 PMID: 25,278,503 PMID: 28,892,042

313

MT000984

Mitochondrial ribosomal protein L18

UniProtKB–Q9H0U6 (RM18_HUMAN), PMID: 25,278,503 PMID: 28,892,042

314

MT001126

Mitochondrial ribosomal protein L20

UniProtKB–Q9BYC9 (RM20_HUMAN), PMID: 11,279,069 PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

315

MT000539

Mitochondrial ribosomal protein L23

UniProtKB–Q16540 (RM23_HUMAN), PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

316

MT001047

Mitochondrial ribosomal protein L27

UniProtKB–Q9P0M9 (RM27_HUMAN), PMID: 11,279,069 PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

317

MT000794

Mitochondrial ribosomal protein L28

UniProtKB–Q13084 (RM28_HUMAN), PMID: 25,278,503 PMID: 28,892,042

318

MT000847

Mitochondrial ribosomal protein L3

UniProtKB–P09001 (RM03_HUMAN), PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

319

MT001285

Mitochondrial ribosomal protein L32

UniProtKB–Q9BYC8 (RM32_HUMAN), PMID: 25,278,503 PMID: 25,838,379 PMID: 28,892,042

320

MT000643

Mitochondrial transcription termination factor 1

UniProtKB–Q99551 (MTEF1_HUMAN)

321

MT001467

Mitochondrial ribosome-associated GTPase 1

UniProtKB–Q9BT17 (MTG1_HUMAN), PMID: 12,808,030 PMID: 23,396,448

322

MT000388

Methylmalonyl CoA mutase

UniProtKB–P22033 (MUTA_HUMAN), PMID: 1,970,180

323

MT001573

N-acetylglutamate synthase

UniProtKB–Q8N159 (NAGS_HUMAN), PMID: 7,126,172

324

MT000403

NADH dehydrogenase (ubiquinone) 1 α subcomplex, 9, 39 kDa

UniProtKB–Q16795 (NDUA9_HUMAN), PMID: 12,611,891

325

MT001652

NOP2/Sun domain family, member 4

UniProtKB–Q96CB9 (NSUN4_HUMAN), PMID: 21,531,335 PMID: 22,949,673

326

MT000445

Ornithine aminotransferase

UniProtKB–P04181 (OAT_HUMAN), PMID: 23,076,989

327

MT000450

Ornithine carbamoyltransferase

UniProtKB–P00480 (OTC_HUMAN)

328

MT000452

3-Oxoacid CoA transferase 1

UniProtKB–P55809 (SCOT1_HUMAN)

329

MT000458

Propionyl CoA carboxylase, α polypeptide

UniProtKB–P05165 (PCCA_HUMAN), PMID: 16,023,992 PMID: 10,101,253

330

MT000459

Propionyl CoA carboxylase, β polypeptide

UniProtKB–P05166 (PCCB_HUMAN), PMID: 16,023,992

331

MT000460

Phosphoenolpyruvate carboxykinase 2 (mitochondrial)

UniProtKB–Q16822 (PCKGM_HUMAN)

332

MT000464

Pyruvate dehydrogenase (lipoamide) β

UniProtKB–P11177 (ODPB_HUMAN)

333

MT000647

Pyruvate dehydrogenase complex, component X

UniProtKB–O00330 (ODPX_HUMAN)

334

MT000467

Pyruvate dehydrogenase kinase, isozyme 3

UniProtKB–Q15120 (PDK3_HUMAN)

335

MT001225

Pyruvate dehydrogenase phosphatase catalytic subunit 2

UniProtKB–Q9P2J9 (PDP2_HUMAN)

336

MT001128

Pyruvate dehydrogenase phosphatase regulatory subunit

UniProtKB–Q8NCN5 (PDPR_HUMAN)

337

MT000898

Prenyl (decaprenyl) diphosphate synthase, subunit 1

UniProtKB–Q5T2R2 (DPS1_HUMAN)

338

MT001210

Prenyl (decaprenyl) diphosphate synthase, subunit 2

UniProtKB–Q86YH6 (DLP1_HUMAN)

339

MT000787

Pitrilysin metallopeptidase 1

UniProtKB–Q5JRX3 (PREP_HUMAN), PMID: 16,849,325 PMID: 19,196,155

340

MT000848

Polymerase (DNA directed), γ 2, accessory subunit

UniProtKB–Q9UHN1 (DPOG2_HUMAN)

341

MT000485

Polymerase (RNA) mitochondrial (DNA directed)

UniProtKB–O00411 (RPOM_HUMAN), PMID: 23,283,301 PMID: 29,445,193

342

MT000932

Pentatricopeptide repeat domain 1

UniProtKB–O75127 (PTCD1_HUMAN), PMID: 19,651,879 PMID: 21,857,155

343

MT001208

Arginyl-tRNA synthetase 2, mitochondrial

UniProtKB–Q5T160 (SYRM_HUMAN)

344

MT001104

Seryl-tRNA synthetase 2, mitochondrial

UniProtKB–Q9NP81 (SYSM_HUMAN)

345

MT000034

Solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4

UniProtKB–P12235 (ADT1_HUMAN)

346

MT000682

Succinate-CoA ligase, ADP-forming, β subunit

UniProtKB–Q9P2R7 (SUCB1_HUMAN), PMID: 15,877,282 PMID: 17,287,286

347

MT000681

Succinate-CoA ligase, α subunit

UniProtKB–P53597 (SUCA_HUMAN)

348

MT000680

Succinate-CoA ligase, GDP-forming, β subunit

UniProtKB–Q96I99 (SUCB2_HUMAN)

349

MT001322

Transcription elongation factor, mitochondrial

UniProtKB–Q96QE5 (TEFM_HUMAN), PMID: 21,278,163

350

MT000593

Transcription factor A, mitochondrial

UniProtKB–Q00059 (TFAM_HUMAN), PMID: 1,737,790

351

MT001258

Transcription factor B2, mitochondrial

UniProtKB–Q9H5Q4 (TFB2M_HUMAN)

352

MT000916

Thioredoxin 2

UniProtKB–Q99757 (THIOM_HUMAN), PMID: 12,032,145 PMID: 12,080,052

353

MT000796

Thioredoxin reductase 2

UniProtKB–Q9NNW7 (TRXR2_HUMAN), PMID: 10,215,850

354

MT000772

Tryptophanyl tRNA synthetase 2, mitochondrial

UniProtKB–Q9UGM6 (SYWM_HUMAN), PMID: 28,236,339

355

MT001021

Tyrosyl-tRNA synthetase 2, mitochondrial

UniProtKB–Q9Y2Z4 (SYYM_HUMAN), PMID: 15,779,907

356

MT000051

ATP synthase, H + transporting, mitochondrial F1 complex, β polypeptide

UniProtKB–P06576 (ATPB_HUMAN)

357

MT000052

ATP synthase, H + transporting, mitochondrial F1 complex, γ polypeptide 1

UniProtKB–P36542 (ATPG_HUMAN)

358

MT000802

Phenylalanyl-tRNA synthetase 2, mitochondrial

UniProtKB–O95363 (SYFM_HUMAN)

359

MT000940

Mitochondrial ribosome-associated GTPase 2

UniProtKB–Q9H4K7 (MTG2_HUMAN)

360

MT000882

Sirtuin 4

UniProtKB–Q9Y6E7 (SIR4_HUMAN)