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. 2020 Jul 8;10(7):1013. doi: 10.3390/biom10071013

Table 2.

Subfamilies of mitochondrial carrier defined by substrate specificity.

Subfamilies Aliases Main Substrates Triplets * References
For nucleotides and dinucleotides
ADP/ATP AAC ADP, ATP 11 (DNS), 19 (AGT), 23 (KL[G/S]), 84 (TYG), 85 (QRX), 88 (NYV) [19,80]
Coenzyme A/PAP CoA/PAP - 23 (K[V/A]Q), 34 (IVR), 88 ([K/Q]SS) [65,81]
ATP-Mg/Pi APC ATP-Mg, ATP-Ca, Pi, AXP 23 (RQ[Q/A]), 30 (DE[A/T/N]), 84 (EYA), 88 (KDS) [19,76,82,83,84]
Thiamine pyrophosphate TPC Thpp, thmp; (d)NDP, (d)NTP 23 (R[T/S]K), 34 (IT[K/R]), 80 (L[A/T]K), 85 (GAT) [85,86,87]
Pyrimidine nucleotides PNC Pyrimidine (deoxy)nucleotides 19 (G[G/A]K), 27 (CNY), 30 ([D/E]WE), 37 (QQR), 83 ([PEP), 85 (R[I/V][S/T]) [48,88]
FAD/folate FAD Folates, FAD 19 (GGK), 27 (HNY), 30 (DWQ) [70,89,90]
ANT ANT ATP, ADP, AMP 19 (SAK), 30 (DAI), 33 (KAK), 37 (QKR) [46]
NAD+ NDT/PXN NAD+, (d)AMP, (d)GMP 19 (GGK), 27 (CNY), 30 (DWE), 89 (FP[L/F]) [49,68]
GTP/GDP GGC GTP, GDP, dgtp, dgdp, ITP, IDP 22 (EGS), 23 (IEL), 84 (QGK), 85 (RSL), 88 (KLS) [91]
For di-/tri-carboxylates and keto acids
Dicarboxylates DIC Malate, succinate, phosphate, sulfate, thiosulfate 26 (TG[C/S]), 27 (H[N/T][S/Q/N]), 33 (K[N/M]K), 88 (RQ[I/L/T]) [42,92,93]
Di-/tri-carboxylates DTC Oxoglutarate, citrate 26 (IGS), 27 (QSL), 33 (KLK), 35 (RRQ), 77 (GTY), 84 (YLH), 88 (RMT), 93 ([K/R]DN) [50]
Citrate/isocitrate SFC Citrate, isocitrate, aconitate 22 (EAG), 84 (KNG), 88 (RNT) [94,95,96]
Citrate CTP Citrate, malate, isocitrate, cis-aconitate, PEP 22 (E[A/S][S/T]), 84 (KN[S/D]), 88 (RRV) [97,98]
2-oxoglutarate OGC 2-Oxoglutarate, malate 26 (VGS), 27 (QTM), 33 (KLK), 35 (RRR), 77 (GTY), 84 (YVH), 88 (RQT), 93 (TSE) [43,99]
Oxodicarboxylates ODC Oxoadipate, oxoglutarate 22 (EE[A/G]), 77 (PTK), 81 (E[H/N]L) 84 (K[F/W]G),
85 (RNG), 88 (KY[M/L])
[100,101]
Oxaloacetate/sulfate OAC Oxaloacetate, sulfate, thiosulfate, a-isopropylmalate 23 (VAA), 26 (TGM), 30 (E[F/Y]D), 80 (YRR), 84 ([L/M]GH), 88 (RQ[C/S]) [47,102]
For amino acids
Glutamate GC Glutamate 22 (GQA), 77 (NTR), 80 (LRV), 84 (EFL), 85 (KSF), 88 (KYA) [103]
Glutamate BOU L-Glutamate - [104]
Aspartate/glutamate AGC Aspartate, glutamate, cysteinesulfinate 22 (GQA), 77 (QCR), 84 (EFQ), 85 (KSF), 88 (KYT) [45,105]
Aspartate/glutamate UCP1–2 23 ([D/E][V/I/S/Q][A/V/T/S]), 88 ([R/K] [D/E][F/M]) [12,51]
Ornithine ORC Ornithine, (lysine, citrulline, arginine, histidine) 23 ([V/I][A/S]W) but (KSN) in S. cerevisiae, 26 (GL[V/C]) but (ELI) in S. cerevisiae, 84 (EGA), but (QAV) in atbac2 [106,107,108]
Carnitine CAC Carnitine, acylcarnitine 23 (VTW), 85 (FSN) [44,109]
S-adenosylmethionine SAMC S-adenosylmethionine, S-adenosylhomocysteine 19 (G[E/G]G), 23 ([D/E][C/S][A/G]), 26 ([L/F]RT), 80 ([G/A]RW), 85 ([A/S][S/T/D]X), 88 (FQF) [110,111,112,113]
For other substrates
Phosphate PiC, mPT Phosphate 19 (CEG), 23 (HDA), 80 (G[R/K]M), 88 (KKQ) [114,115]
Iron MIT, MRS–4, MFRN–2 - 19 (GTG), 22 (E[S/A/H][A/C]), 23 (HDA), 27 ([F/Y][T/N]T) [12,116]

Abbreviations: AAC, ADP/ATP carrier; AGC, aspartate/glutamate carrier; ANT, peroxisomal adenine nucleotide translocator; APC, ATP-Mg/Pi carrier; CAC, carnitine carrier; CoA/PAP, coenzyme A/adenosine 3’,5’-diphosphate carrier; BOU, A bout de soufflé (glutamate transporter); CTP, citrate carrier; DIC, dicarboxylate carrier; DTC, di-/tri-carboxylate carrier; FAD, FAD carrier; GC, glutamate carrier, GGC, GTP/GDP carrier; NDT, NAD+ carrier; OAC, oxaloacetate/sulfate carrier; ODC, oxodicarboxylate carrier; OGC, oxoglutarate carrier; ORC, ornithine carrier; PiC, phosphate carrier; mPT, mitochondrial phosphate carrier; PNC, pyrimidine nucleotide carrier; SAMC, S-adenosylmethionine carrier; SFC, succinate/fumarate carrier; TPC, thiamine pyrophosphate carrier; UCP, uncoupling protein. AXP, adenine nucleotides; dNDP, deoxynucleoside diphosphates; dNTP, deoxynucleoside triphosphates; PEP, phosphoenolpyruvate; Pi, phosphate; ThMP, thiamine monophosphate; ThPP, thiamine pyrophosphate. * Symmetry-related amino acid triplets are the triplet sets present in the functionally identified mitochondrial carriers of each family.