Label Type Identifier Comment Ensembl NCBI gene HGNC UniProt Wikidata ChEBI InChI PubChem ChemSpider HMDB KEGG LipidMaps [4Fe-4A]clusters Metabolite chebi:64607 "" wikidata:Q75078829 chebi:64607 MT-ND5 Protein uniprot:P03915 "67 KD" ensembl:ENSG00000198786 ncbigene:4540 hgnc.symbol:MT-ND5 uniprot:U5ZC31;uniprot:P03915 NDUFB2 Protein uniprot:O95178 "12 KD" ensembl:ENSG00000090266 ncbigene:4708 hgnc.symbol:NDUFB2 uniprot:H7C5B8;uniprot:C9IZZ2;uniprot:C9JRV4;uniprot:C9JXM4;uniprot:A4D1T5;uniprot:C9IZW8;uniprot:F8WCJ6;uniprot:O95178 NDUFB3 Protein uniprot:O43676 "~12 KD" ensembl:ENSG00000119013 ncbigene:4709 hgnc.symbol:NDUFB3 uniprot:O43676;uniprot:C9JKQ2;uniprot:A0A024R413 NDUFB7 Protein uniprot:P17568 "16.4 KD" ensembl:ENSG00000099795 ncbigene:4713 hgnc.symbol:NDUFB7 uniprot:P17568;uniprot:M0R3B8 NDUFB8 Protein uniprot:O95169 "22 KD" ensembl:ENSG00000166136 ncbigene:4714 hgnc.symbol:NDUFB8 uniprot:E9PIZ8;uniprot:O95169 NDUFB9 Protein uniprot:Q9Y6M9 "22 KD" ensembl:ENSG00000147684 ncbigene:4715 hgnc.symbol:NDUFB9 uniprot:E7EWZ0;uniprot:A0A7I2V620;uniprot:E9PH64;uniprot:E9PF49;uniprot:A0A3B3IT57;uniprot:Q9Y6M9;uniprot:A0A7I2YQN0 NDUFAB1 Protein uniprot:O14561 "~16 KD" ensembl:ENSG00000004779 ncbigene:4706 hgnc.symbol:NDUFAB1 uniprot:I3L505;uniprot:O14561;uniprot:H3BNK3;uniprot:H3BNE8 DMAC1 Protein uniprot:Q96GE9 "DMAC1/TMEM261 is implicated in the stabilization and/or assembly of the ND5-module
11,8 KD" ensembl:ENSG00000137038 ncbigene:90871 hgnc.symbol:DMAC1 uniprot:Q96GE9 NDUFAF2 Protein uniprot:Q8N183 "Stabilising intermediated only lacking the N-module" ensembl:ENSG00000164182 ncbigene:91942 hgnc.symbol:NDUFAF2 uniprot:Q8N183;uniprot:A0A7I2V4Z7;uniprot:A0A7I2YQX2;uniprot:H0YA50;uniprot:D6RA56;uniprot:A0A0S2Z5U1;uniprot:A0A7I2V3X5 NDUFS3 Protein uniprot:O75489 "30.2 KDa (protein atlas)" ensembl:ENSG00000213619 ncbigene:4722 hgnc.symbol:NDUFS3 uniprot:E9PKL8;uniprot:E9PS48;uniprot:G3V194;uniprot:E9PJE7;uniprot:O75489 NDUFB5 Protein uniprot:O43674 "" ensembl:ENSG00000136521 ncbigene:4711 hgnc.symbol:NDUFB5 uniprot:O43674;uniprot:H7C568;uniprot:F8WDZ2;uniprot:A0A087WYD0;uniprot:H7C5C1;uniprot:H0Y886;uniprot:E7EWP0;uniprot:F8WDD2 TIMMDC1 Protein uniprot:Q9NPL8 "aka C3ORF1
Chaperone
remains bound to the Q/ND1 subassembly until the last maturation steps." ensembl:ENSG00000113845 ncbigene:51300 hgnc.symbol:TIMMDC1 uniprot:Q9NPL8;uniprot:F8WC42;uniprot:C9JR82;uniprot:H7C5U1;uniprot:G3XA94;uniprot:C9JU35;uniprot:F8WAU6;uniprot:F8WB00 MT-ND4 Protein uniprot:P03905 "52 Kda" ensembl:ENSG00000198886 ncbigene:4538 hgnc.symbol:MT-ND4 uniprot:H9EC08;uniprot:P03905 NDUFAF3 Protein uniprot:Q9BU61 "Chaperone" ensembl:ENSG00000178057 ncbigene:25915 hgnc.symbol:NDUFAF3 uniprot:A4FU71;uniprot:Q9BU61 FOXRED1 Protein uniprot:Q96CU9 "" ensembl:ENSG00000110074 ncbigene:55572 hgnc.symbol:FOXRED1 uniprot:A0A8I5KXP7;uniprot:Q96CU9;uniprot:A0A8I5KRD2;uniprot:A0A8I5KY79;uniprot:A0A8I5KZ12;uniprot:A0A8I5KPI4;uniprot:A0A8I5KSK3;uniprot:A0A8I5KUS8;uniprot:A0A8I5KU12;uniprot:A0A8I5KQQ8;uniprot:A0A8I5KTW5;uniprot:E9PP78;uniprot:E9PNK9;uniprot:A0A8I5KU90;uniprot:A0A8I5QL46;uniprot:A0A8J9AK24 NDUFV1 Protein uniprot:P49821 "" ensembl:ENSG00000167792 ncbigene:4723 hgnc.symbol:NDUFV1 uniprot:E9PMX3;uniprot:H0YE81;uniprot:E9PPR0;uniprot:H0YD04;uniprot:E9PPS5;uniprot:E9PQP1;uniprot:P49821;uniprot:E5KNH5;uniprot:E9PJL9;uniprot:G3V0I5;uniprot:B4DE93;uniprot:E9PPE0;uniprot:E9PLC6;uniprot:E9PPD6 NDUFA5 Protein uniprot:Q16718 "13.5 KD" ensembl:ENSG00000128609 ncbigene:4698 hgnc.symbol:NDUFA5 uniprot:C9IZN5;uniprot:Q16718;uniprot:A0A087WXR5;uniprot:A0A024R772;uniprot:H7BYD0;uniprot:F8WAS3;uniprot:A0A024R745;uniprot:A0A087X1G1;uniprot:A0A7I2V2W4;uniprot:A0A7I2V3L8 MT-ND1 Protein uniprot:P03886 "" ensembl:ENSG00000198888 ncbigene:4535 hgnc.symbol:MT-ND1 uniprot:P03886;uniprot:U5Z754 NDUFA1 Protein uniprot:O15239 "8.1 KD" ensembl:ENSG00000125356 ncbigene:4694 hgnc.symbol:NDUFA1 uniprot:Q6IBB5;uniprot:O15239 COA1 Protein uniprot:Q9GZY4 "Chaperone" ensembl:ENSG00000106603 ncbigene:55744 hgnc.symbol:COA1 uniprot:A0A024RA60;uniprot:C9J6J1;uniprot:Q9GZY4;uniprot:F8WBS2;uniprot:F2Z2J3;uniprot:C9JA07 MT-ND3 Protein uniprot:Q9GZY4 "" ensembl:ENSG00000106603 ncbigene:55744 hgnc.symbol:COA1 uniprot:A0A024RA60;uniprot:C9J6J1;uniprot:Q9GZY4;uniprot:F8WBS2;uniprot:F2Z2J3;uniprot:C9JA07 TMEM126B Protein uniprot:Q8IUX1 "Not in Figure at first step, but written down in text!" ensembl:ENSG00000171204 ncbigene:55863 hgnc.symbol:TMEM126B uniprot:E9PKZ7;uniprot:A0A024R5L4;uniprot:H0YD74;uniprot:E9PKZ9;uniprot:E9PJQ6;uniprot:Q8IUX1;uniprot:H0YEG5 NDUFS1 Protein uniprot:P28331 "" ensembl:ENSG00000023228 ncbigene:4719 hgnc.symbol:NDUFS1 uniprot:C9JPQ5;uniprot:F8WDL5;uniprot:P28331;uniprot:B4DJ81;uniprot:E5KRK5 TMEM186 Protein uniprot:Q96B77 "Chaperone" ensembl:ENSG00000184857 ncbigene:25880 hgnc.symbol:TMEM186 uniprot:Q96B77 NDUFA6 Protein uniprot:P56556 "" ensembl:ENSG00000184983 ncbigene:4700 hgnc.symbol:NDUFA6 uniprot:R4GN43;uniprot:P56556;uniprot:A0A2Y9D025 NDUFA3 Protein uniprot:O95167 "9.3 KD" ensembl:ENSG00000170906 ncbigene:4696 hgnc.symbol:NDUFA3 uniprot:A8MUI2;uniprot:S4R3I5;uniprot:F2Z2C6;uniprot:A8MYJ8;uniprot:Q6FGG4;uniprot:H7C0D7;uniprot:H7C2R1;uniprot:O95167;uniprot:S4R3T6;uniprot:A8MVM8 NDUFV2 Protein uniprot:P19404 "" ensembl:ENSG00000178127 ncbigene:4729 hgnc.symbol:NDUFV2 uniprot:E7EPT4;uniprot:J3QS34;uniprot:J3KRB4;uniprot:P19404 NDUFA2 Protein uniprot:O43678 "" ensembl:ENSG00000131495 ncbigene:4695 hgnc.symbol:NDUFA2 uniprot:O43678 NDUFA7 Protein uniprot:O95182 "" ensembl:ENSG00000267855 ncbigene:4701 hgnc.symbol:NDUFA7 uniprot:M0R0N0;uniprot:O95182;uniprot:M0R1K9 NDUFA12 Protein uniprot:Q9UI09 "" ensembl:ENSG00000184752 ncbigene:55967 hgnc.symbol:NDUFA12 uniprot:A0A804HJF8;uniprot:F8VRD8;uniprot:Q9UI09;uniprot:H0YID5;uniprot:A0A804HHX9;uniprot:F8VXI1;uniprot:H0YIT1;uniprot:A0A804HK60 NDUFS4 Protein uniprot:O43181 "" ensembl:ENSG00000164258 ncbigene:4724 hgnc.symbol:NDUFS4 uniprot:D6R916;uniprot:A0A0S2Z433;uniprot:D6RI09;uniprot:O43181;uniprot:D6REP1;uniprot:H0Y9M8 NDUFS6 Protein uniprot:O75380 "" ensembl:ENSG00000145494 ncbigene:4726 hgnc.symbol:NDUFS6 uniprot:Q6IBC4;uniprot:D6RBT3;uniprot:O75380 NDUFV3 Protein uniprot:P56181 "" ensembl:ENSG00000160194 ncbigene:4731 hgnc.symbol:NDUFV3 uniprot:P56181 NDUFB10 Protein uniprot:O96000 "" ensembl:ENSG00000140990 ncbigene:4716 hgnc.symbol:NDUFB10 uniprot:H3BPJ9;uniprot:A8K761;uniprot:O96000;uniprot:H3BV16 NDUFB11 Protein uniprot:Q9NX14 "" ensembl:ENSG00000147123 ncbigene:54539 hgnc.symbol:NDUFB11 uniprot:A0A8I5KTD1;uniprot:Q9NX14;uniprot:A0A8J8YU24;uniprot:A0A8I5KQF7;uniprot:A0A8I5KTJ9 NDUFB6 Protein uniprot:O95139 "" ensembl:ENSG00000165264 ncbigene:4712 hgnc.symbol:NDUFB6 uniprot:O95139;uniprot:A0A087WZX2 NDUFB1 Protein uniprot:O75438 "7 KD" ensembl:ENSG00000183648 ncbigene:4707 hgnc.symbol:NDUFB1 uniprot:A0A024R6G6;uniprot:O75438 NDUFB4 Protein uniprot:O95168 "15 Kd
Not believed to be involved in catalysis" ensembl:ENSG00000065518 ncbigene:4710 hgnc.symbol:NDUFB4 uniprot:C9JXQ9;uniprot:O95168;uniprot:F2Z3P9 ATP5SL Protein uniprot:Q9NW81 "" ensembl:ENSG00000105341 ncbigene:55101 hgnc.symbol:DMAC2 uniprot:M0QX81;uniprot:M0QZP7;uniprot:M0R078;uniprot:K7ESN5;uniprot:K7EIV4;uniprot:Q9NW81;uniprot:B4DFT4;uniprot:A0A024R0K4;uniprot:M0QZC4 TMEM70 Protein uniprot:Q9BUB7 "aka cV assembly factor" ensembl:ENSG00000175606 ncbigene:54968 hgnc.symbol:TMEM70 uniprot:Q9BUB7;uniprot:D4PHA6 MTND2 Protein uniprot:P03891 "" ensembl:ENSG00000198763 ncbigene:4536 hgnc.symbol:MT-ND2 uniprot:Q7GXY9;uniprot:P03891 NDUFC1 Protein uniprot:O43677 "" ensembl:ENSG00000109390 ncbigene:4717 hgnc.symbol:NDUFC1 uniprot:O43677;uniprot:A0A6Q8PHK7 NDUFC2 Protein uniprot:O95298 "" ensembl:ENSG00000151366 ncbigene:4718 hgnc.symbol:NDUFC2 uniprot:A0A024R5K6;uniprot:O95298;uniprot:E9PM14;uniprot:E9PRJ5 NDUFAF1 Protein uniprot:Q9Y375 "aka CIA30" ensembl:ENSG00000137806 ncbigene:51103 hgnc.symbol:NDUFAF1 uniprot:A0A7I2V4I7;uniprot:A0A024R9L0;uniprot:A0A7I2YQA4;uniprot:H0YL22;uniprot:Q9Y375;uniprot:H0YNB7;uniprot:H0YNN4 ECSIT Protein uniprot:Q9BQ95 "" ensembl:ENSG00000130159 ncbigene:51295 hgnc.symbol:ECSIT uniprot:A0A8I5KR62;uniprot:K7ESM8;uniprot:K7EIK2;uniprot:K7EM98;uniprot:K7EN32;uniprot:K7EPL5;uniprot:J3KTF5;uniprot:K7EJG5;uniprot:K7EJI1;uniprot:Q9BQ95 ACAD9 Protein uniprot:Q9H845 "" ensembl:ENSG00000177646 ncbigene:28976 hgnc.symbol:ACAD9 uniprot:Q9H845;uniprot:A0A7P0T9N9;uniprot:A0A7P0TB68;uniprot:D6R9Z3;uniprot:D6RDK9;uniprot:D6RGK6;uniprot:A0A7P0TB58;uniprot:D6RCD8;uniprot:A0A7P0T7Z1;uniprot:Q9H9W4;uniprot:H0Y8Z9;uniprot:A0A7P0Z450;uniprot:A0A7P0T8U3;uniprot:D6RJA8;uniprot:A0A7P0T871 MT-ND6 Protein uniprot:P03923 "18 KD" ensembl:ENSG00000198695 ncbigene:4541 hgnc.symbol:MT-ND6 uniprot:P03923;uniprot:U5Z977 MT-ND4L Protein uniprot:P03901 "11 KD" ensembl:ENSG00000212907 ncbigene:4539 hgnc.symbol:MT-ND4L uniprot:Q7GXZ4;uniprot:P03901 NDUFAF1 Protein uniprot:Q9Y375 "" ensembl:ENSG00000137806 ncbigene:51103 hgnc.symbol:NDUFAF1 uniprot:A0A7I2V4I7;uniprot:A0A024R9L0;uniprot:A0A7I2YQA4;uniprot:H0YL22;uniprot:Q9Y375;uniprot:H0YNB7;uniprot:H0YNN4 NDUFA10 Protein uniprot:O95299 "41KD" ensembl:ENSG00000130414 ncbigene:4705 hgnc.symbol:NDUFA10 uniprot:A0A7I2V2F6;uniprot:A0A7I2V3D4;uniprot:A0A7I2V5B2;uniprot:A0A7I2YQU0;uniprot:H7C2X4;uniprot:A0A7I2V2W9;uniprot:A0A7I2V2Q2;uniprot:A0A7I2V327;uniprot:A0A7I2V5U7;uniprot:A0A7I2V2I6;uniprot:C9J6X0;uniprot:H7C1Y7;uniprot:A0A087WXC5;uniprot:A0A7I2V2X3;uniprot:H7C2W5;uniprot:A0A7I2V594;uniprot:F8WEH0;uniprot:Q8N1B9;uniprot:E7ESZ7;uniprot:A0A7I2V458;uniprot:A0A7I2V2N6;uniprot:A0A7I2V438;uniprot:A0A7I2V5L0;uniprot:A0A7I2V419;uniprot:O95299;uniprot:A0A7I2V2J5;uniprot:A0A7I2V4N8;uniprot:A0A7I2V3H5 NDUFS5 Protein uniprot:O43920 "15KD" ensembl:ENSG00000168653 ncbigene:4725 hgnc.symbol:NDUFS5 uniprot:Q6IBA0;uniprot:O43920 TMEM186 Protein uniprot:Q96B77 "" ensembl:ENSG00000184857 ncbigene:25880 hgnc.symbol:TMEM186 uniprot:Q96B77 COA1 Protein uniprot:Q9GZY4 "" ensembl:ENSG00000106603 ncbigene:55744 hgnc.symbol:COA1 uniprot:A0A024RA60;uniprot:C9J6J1;uniprot:Q9GZY4;uniprot:F8WBS2;uniprot:F2Z2J3;uniprot:C9JA07 NDUFAF6 Protein uniprot:Q330K2 "38.2 kDa
seems to participate in the assembly of the Q-module
necessary to maintain normal MT-ND1 synthesis" ensembl:ENSG00000156170 ncbigene:137682 hgnc.symbol:NDUFAF6 uniprot:H0YC61;uniprot:E5RGD6;uniprot:E5RHX9;uniprot:H0YBQ9;uniprot:Q330K2;uniprot:A0A075B6P0;uniprot:E5RFN5;uniprot:H0YB05;uniprot:H0YBT9;uniprot:H7C250;uniprot:A0A8V8TLC1;uniprot:H0YB46 NUBPL Protein uniprot:Q8TB37 "" ensembl:ENSG00000151413 ncbigene:80224 hgnc.symbol:NUBPL uniprot:F8W061;uniprot:H0YHR7;uniprot:F8VZR8;uniprot:F8W0A2;uniprot:X5D2R5;uniprot:F8VP02;uniprot:Q8TB37 NDUFS2 Protein uniprot:O75306 "49 KD" ensembl:ENSG00000158864 ncbigene:4720 hgnc.symbol:NDUFS2 uniprot:A0A7I2V4G5;uniprot:A0A7I2V5E2;uniprot:A0A7I2V5G4;uniprot:A0A7I2YQD8;uniprot:A0A7I2V5D5;uniprot:A0A7I2V2B9;uniprot:O75306;uniprot:A0A7I2YQX7;uniprot:A0A7I2V4W5;uniprot:A0A7I2V596;uniprot:A0A7I2V4X8;uniprot:A0A7I2V5X7;uniprot:A0A7I2V2P1;uniprot:A0A7I2V410;uniprot:A0A7I2V630;uniprot:A0A7I2V517;uniprot:A0A7I2V3J6;uniprot:A0A7I2YQG7 NDUFAF7 Protein uniprot:Q7L592 "49.2 kD (proteinatlas)" ensembl:ENSG00000003509 ncbigene:55471 hgnc.symbol:NDUFAF7 uniprot:Q7L592;uniprot:B4E0W7;uniprot:C9JP36;uniprot:H7C3N5;uniprot:C9JEL7;uniprot:C9J236;uniprot:C9JS27 NDUFS8 Protein uniprot:O "23,7 KD
The encoded protein(TYKY) contains two [4Fe-4S] ferredoxin consensus patterns [PMID: 9837812]" NDUFAF5 Protein uniprot:Q "" NDUFS7 Protein uniprot:O "20 kD" NDUFS7 Protein uniprot:O "" NDUFAF4 Protein uniprot:Q9P032 "Chaperone" ensembl:ENSG00000123545 ncbigene:29078 hgnc.symbol:NDUFAF4 uniprot:Q9P032 NDUFAF3 Protein uniprot:Q9BU61 "Chaperone
Remain bound to this module until the final assembly steps" ensembl:ENSG00000178057 ncbigene:25915 hgnc.symbol:NDUFAF3 uniprot:A4FU71;uniprot:Q9BU61 NDUFAF4 Protein uniprot:Q9P032 "Chaperone
Remain bound to this module until the final assembly steps" ensembl:ENSG00000123545 ncbigene:29078 hgnc.symbol:NDUFAF4 uniprot:Q9P032 NDUFA8 Protein uniprot:P51970 "20 kD" ensembl:ENSG00000119421 ncbigene:4702 hgnc.symbol:NDUFA8 uniprot:P51970 NDUFA13 Protein uniprot:Q9P0J0 "17 kD" ensembl:ENSG00000186010 ncbigene:51079 hgnc.symbol:NDUFA13 uniprot:K7EP87;uniprot:B4DEZ3;uniprot:U3KQP3;uniprot:K7EJE1;uniprot:Q9P0J0