# STOCKHOLM 1.0 #=GF ID NDUF_B7 #=GF AC PF05676.16 #=GF DE NADH-ubiquinone oxidoreductase B18 subunit (NDUFB7) #=GF PI NDUFB7; #=GF AU Moxon SJ;0000-0003-4644-1816 #=GF SE Pfam-B_7077 (release 8.0) #=GF GA 26.00 26.00; #=GF TC 26.10 26.00; #=GF NC 25.70 25.90; #=GF BM hmmbuild HMM.ann SEED.ann #=GF SM hmmsearch -Z 61295632 -E 1000 --cpu 4 HMM pfamseq #=GF TP Family #=GF WK NADH_dehydrogenase_(ubiquinone) #=GF CL CL0351 #=GF RN [1] #=GF RM 10830904 #=GF RT Characterization of the human complex I NDUFB7 and 17.2-kDa #=GF RT cDNAs and mutational analysis of 19 genes of the HP fraction in #=GF RT complex I-deficient-patients. #=GF RA Triepels R, Smeitink J, Loeffen J, Smeets R, Trijbels F, van den #=GF RA Heuvel L; #=GF RL Hum Genet 2000;106:385-391. #=GF DR INTERPRO; IPR008698; #=GF DR SO; 0100021; polypeptide_conserved_region; #=GF CC This family consists of several NADH-ubiquinone oxidoreductase #=GF CC B18 subunit proteins from different eukaryotic organisms. #=GF CC Oxidative phosphorylation is the well-characterised process in #=GF CC which ATP, the principal carrier of chemical energy of #=GF CC individual cells, is produced due to a mitochondrial proton #=GF CC gradient formed by the transfer of electrons from NADH and FADH2 #=GF CC to molecular oxygen. The oxidative phosphorylation (OXPHOS) #=GF CC system is located in the mitochondrial inner membrane and #=GF CC consists of five multi-subunit enzyme complexes and two small #=GF CC electron carriers: coenzyme Q10 and cytochrome C. At least 70 #=GF CC structural proteins involved in the formation of the whole #=GF CC OXPHOS system are encoded by nuclear genes, whereas 13 #=GF CC structural proteins are encoded by the mitochondrial genome. #=GF CC Deficiency of NADH ubiquinone oxidoreductase, the first enzyme #=GF CC complex of the mitochondrial respiratory chain, is one of the #=GF CC most frequent causes of human mitochondrial encephalomyopathies #=GF CC [1]. #=GF SQ 70 #=GS F4S8P7_MELLP/7-68 AC F4S8P7.1 #=GS M9M7H2_PSEA3/6-68 AC M9M7H2.1 #=GS U5HH20_USTV1/17-75 AC U5HH20.1 #=GS G4T9D7_SERID/8-70 AC G4T9D7.1 #=GS M5BVS5_THACB/5-67 AC M5BVS5.1 #=GS I4YGC2_WALMC/5-66 AC I4YGC2.1 #=GS D8QHL6_SCHCM/6-67 AC D8QHL6.1 #=GS K5W541_PHACS/6-71 AC K5W541.1 #=GS V2WNT4_MONRO/5-65 AC V2WNT4.1 #=GS B0DBA8_LACBS/6-68 AC B0DBA8.1 #=GS A8NC29_COPC7/6-68 AC A8NC29.1 #=GS Q5KK26_CRYNJ/6-68 AC Q5KK26.1 #=GS A5BAM7_VITVI/11-73 AC A5BAM7.1 #=GS A0A0D9Z1L7_9ORYZ/19-81 AC A0A0D9Z1L7.1 #=GS A9SE49_PHYPA/8-70 AC A9SE49.1 #=GS D8T1P1_SELML/2-63 AC D8T1P1.1 #=GS G1X8L5_ARTOA/9-71 AC G1X8L5.1 #=GS N1J7Q8_BLUG1/19-81 AC N1J7Q8.1 #=GS N1Q7N9_PSEFD/26-88 AC N1Q7N9.1 #=GS M2NHQ1_BAUPA/20-82 AC M2NHQ1.1 #=GS G0SAE9_CHATD/11-73 AC G0SAE9.1 #=GS C8VIG1_EMENI/17-79 AC C8VIG1.1 #=GS A0A0C4EA84_MAGP6/11-73 AC A0A0C4EA84.1 #=GS I1C3R9_RHIO9/5-66 AC I1C3R9.1 #=GS D0NJK1_PHYIT/11-73 AC D0NJK1.1 #=GS R4X7U4_TAPDE/13-74 AC R4X7U4.1 #=GS A0A1X7U8Y8_AMPQE/10-71 AC A0A1X7U8Y8.1 #=GS A7RTS0_NEMVE/9-71 AC A7RTS0.1 #=GS C3YCJ2_BRAFL/44-106 AC C3YCJ2.1 #=GS A0A7M7RBD0_STRPU/40-102 AC A0A7M7RBD0.1 #=GS A0A7M7G7M5_APIME/38-100 AC A0A7M7G7M5.1 #=GS E2C5R3_HARSA/2-64 AC E2C5R3.1 #=GS E1ZZG8_CAMFO/2-64 AC E1ZZG8.1 #=GS A0A158NN77_ATTCE/40-102 AC A0A158NN77.1 #=GS A0A7M6W5R8_NASVI/42-104 AC A0A7M6W5R8.1 #=GS J9JJU0_ACYPI/39-101 AC J9JJU0.1 #=GS T1JWJ7_TETUR/45-107 AC T1JWJ7.1 #=GS D6X169_TRICA/47-109 AC D6X169.1 #=GS E9G263_DAPPU/1-61 AC E9G263.1 #=GS T1HHP5_RHOPR/42-104 AC T1HHP5.1 #=GS B7PUG2_IXOSC/47-109 AC B7PUG2.1 #=GS A0A212F1I6_DANPL/39-101 AC A0A212F1I6.1 #=GS W5J798_ANODA/40-102 AC W5J798.1 #=GS Q29II4_DROPS/38-100 AC Q29II4.1 #=GS F7BC32_CIOIN/36-98 AC F7BC32.1 #=GS G4LVB0_SCHMA/39-101 AC G4LVB0.1 #=GS F7G4X2_MONDO/40-102 AC F7G4X2.1 #=GS G3HD23_CRIGR/40-102 AC G3HD23.1 #=GS M4ACV7_XIPMA/40-102 AC M4ACV7.2 #=GS A4II52_XENTR/35-97 AC A4II52.1 #=GS Q6P6E5_DANRE/40-102 AC Q6P6E5.1 #=GS E5STE0_TRISP/43-106 AC E5STE0.1 #=GS E0VEI7_PEDHC/681-743 AC E0VEI7.1 #=GS A0A2H2IDU2_CAEJA/43-106 AC A0A2H2IDU2.1 #=GS G8B8H3_CANPC/13-71 AC G8B8H3.1 #=GS B5RTW7_DEBHA/10-72 AC B5RTW7.1 #=GS G3AFU7_SPAPN/10-72 AC G3AFU7.1 #=GS G3BDT0_CANTC/11-71 AC G3BDT0.1 #=GS F4P9U9_BATDJ/2-64 AC F4P9U9.1 #=GS K0KJA2_WICCF/9-68 AC K0KJA2.1 #=GS B5FVG1_YARLI/8-69 AC B5FVG1.1 #=GS B5FVG1_YARLI/8-69 DR PDB; 6Y79 8; 7-69; #=GS B5FVG1_YARLI/8-69 DR PDB; 6YJ4 o; 7-69; #=GS C4QWB4_KOMPG/37-99 AC C4QWB4.1 #=GS C1N513_MICPC/9-70 AC C1N513.1 #=GS A4RRY0_OSTLU/10-72 AC A4RRY0.1 #=GS D7FZ56_ECTSI/11-72 AC D7FZ56.1 #=GS Q6UKY7_CHLRE/11-72 AC Q6UKY7.1 #=GS E1ZNP8_CHLVA/685-745 AC E1ZNP8.1 #=GS D3BII5_POLPP/18-75 AC D3BII5.1 #=GS NDUB7_DICDI/19-77 AC Q54V61.1 #=GS F4PPP8_CAVFA/20-81 AC F4PPP8.1 F4S8P7_MELLP/7-68 .ASQEDMKKARLPLGYRDGCANYLITLNKCRHKEYF.MPWKCGDERHAYEHC...QYKDYVQRMKKM M9M7H2_PSEA3/6-68 TASVDEANAARLPLGYRDQCSALLIPLNKCRKQTLY.APWKCEDERHTYEKC...QYDDFIRRQREL U5HH20_USTV1/17-75 ....ELANRARLPIGWRDQCGKLLIPLNVCRHENLY.MTWKCDHEKHVYEKC...QYDDYMNRMKQL G4T9D7_SERID/8-70 TASQAEATKAHLPIGFRDHCSALLIPLNMCRRENKF.VPWQCDHERHEYEKC...QYDDLMARMKEL M5BVS5_THACB/5-67 IASQQDAAKARLPLAYRDQCSALLIPLNKCRRQGNY.MQWNCEHERHEYEKC...QYDDLLRRMKQL I4YGC2_WALMC/5-66 VASKEECMKNRVPLNWRDQCSGLLIPLNVCRHKNLY.APWACTDERHGYEKC...QYDDMMRRMKV. D8QHL6_SCHCM/6-67 TASEAEMKAAKLPLGWRDQCSALLIPLNVCRKKSFY.LPWECEHERHSYEKC...QYDDYMRRMKA. K5W541_PHACS/6-71 TASQEELKDAKVPLGWRDRCSALLIPLNVCRREKSY.LPWECEDQKHAYEKCVCVAHSLYVRRMKAL V2WNT4_MONRO/5-65 LATQEEMKKAQMPLGWRDQCSALLIPLNVCRREKNY.LPWECEHEKHAYEKC...QYDDYVRRMT.. B0DBA8_LACBS/6-68 TASQEELKAHRVPLAWRDQCSSLLIPLNLCRKQKLY.KPWECEHERHTYDKC...QYDDYVRRMKEL A8NC29_COPC7/6-68 TLSQEEMKAHRIPLGWRDNCSALLVPLNICRRKNNF.KPWECDHERHTYEKC...QYDDYVRRMKEL Q5KK26_CRYNJ/6-68 TASQTEMEAARVPLGWRDQCSALLIPLNVCRHKELY.MPWKCEDERHGYEKC...QYDDYIRRMKQL A5BAM7_VITVI/11-73 IATQEEMVEAKVPLGYRDQCAHLLIPLNKCRHAEFY.LPWKCEAERHVYEKC...EYELVMERMLKM A0A0D9Z1L7_9ORYZ/19-81 IATQAEMSEARLPLPYRDQCAHLLIPLNKCRVAEYY.LPWKCEPERHAYEKC...QYELVMERMIQM A9SE49_PHYPA/8-70 LATQEEMVEARLPLGSRDMCSKLLIPLNKCRQDTFY.LPWKCEDERHGYEKC...EYDLFLQRMKKM D8T1P1_SELML/2-63 .ASNKEMERARVPLEWRDQCAHRLIPLNKCRQQNYY.LPWRCVELRHSFEKC...QYYLYLARVWKM G1X8L5_ARTOA/9-71 VVTAEQMSEARLPIAYRDRCAGLLIPLNRCRFETMY.LPWKCENERHSYEKC...QYDEFMKRVKKM N1J7Q8_BLUG1/19-81 SASREEMSQARLPIQYRDGCAGLLIPLNRCRFKEYY.LPWKCENERHSYEKC...QYDEFLMRVAKM N1Q7N9_PSEFD/26-88 QATRKEMSDAKLPLAYRDSCAHLLIPLNKCRFDNYY.LPWRCMDERHGYEKC...QYEEFKLRVKKM M2NHQ1_BAUPA/20-82 EATREEMSAARVPLPYRDSCAHLLIPLNKCRYDNYF.MPWTCMDERHSYEKC...QYDEFKLRVKKM G0SAE9_CHATD/11-73 RATREEMRDAKVPLAYRDSCAHLLIPLNRCRYETYY.LPWKCEDERHSYEKC...QYLEFKKRVQKM C8VIG1_EMENI/17-79 QATREEMSAARLPLQYRDSCAHLLIPLNRCRQQEWY.LPWKCEDERHSYEKC...QYEEFKKRVAKM A0A0C4EA84_MAGP6/11-73 KATRDEMRDANLPIQYRDTCAHLLIPLNRCRSATYY.LPWKCEDERHSYEKC...QYDEFKLRVAKM I1C3R9_RHIO9/5-66 KATQKDMAKSRIPLEYRDYCANLLIPLNKCRGETFY.LPWKCENERHAYEKC...QYDDFKRRMKE. D0NJK1_PHYIT/11-73 VPTRQEMVDAKLPLHYRDTCAGLLIPLNECRRATFF.LPWKCQDLRHAYEKC...QYEEWKTRVEHL R4X7U4_TAPDE/13-74 .PSQEAMAQAKLPLGYRDNCAHLLIPLNKCRSATFS.LPYKCEDERHSYEKC...QYEDFKKRVKEF A0A1X7U8Y8_AMPQE/10-71 KVTQEEMAEAKVPLNLRDYCAHHFIPLMKCRKDNYY.LPWRCKKEKHAWDEC...EFQDYLDRVRQ. A7RTS0_NEMVE/9-71 KVTAQEMADAKLPLHARDYCAHLLIPLMKCRKDTYY.VPWKCHHEKHAWDKC...EYDDFMYRVREK C3YCJ2_BRAFL/44-106 VATQKEMADARVPMQLRDYCAHKYMAWMMCRRDHMP.NLWACKHERHEWDQC...EYDDWVHRMKEW A0A7M7RBD0_STRPU/40-102 VASQEEMNMARVPMDKRDFCAHFYIAFLKCRRDNFP.NLLNCRHAKHEYDHC...EYEDFVIRMKEY A0A7M7G7M5_APIME/38-100 PLSEEEMIAAKIPHDLRDYCAHYYLDYIRCYMEKFP.FVTRCVTEIHNYQKC...EYDDYILRGKEY E2C5R3_HARSA/2-64 LVSEEDMIAAKIPLNKRDFCAHKYMDLLECQRHHYP.FMLKCAHEKHEYHHC...LADDAVLRLKEY E1ZZG8_CAMFO/2-64 KVSEEDMIAARIPLKQRDYCAHKYLELHDCKRIHYP.FMIKCAHKRHEYYEC...YVEDYVLRMKEY A0A158NN77_ATTCE/40-102 PLSEEDMMAFKIPYDRRDYCAHKYMELHECKRRHYP.FMLKCAHERHQYNEC...YVEDYVIRMKEY A0A7M6W5R8_NASVI/42-104 VAKEEELRAAKIPKDKWDFCAHTLLEVERCRADHFP.FVYRCKEQAHAASMC...NLEDYLLRMKEY J9JJU0_ACYPI/39-101 IASLAEMESAKVPLDARDFCAHMLLNLRGCIRDNFP.FNHHCHHEREEYYEC...QYHDYLDRMKDY T1JWJ7_TETUR/45-107 LATEAEMESAGLPDEKRDYCAHWVIKYDACVRDNLP.FLRTCSYYKEGWRDC...QYNDMVLRMKEY D6X169_TRICA/47-109 IATEEEMRSAKIPLEHRDYCAHHLLKYLSCRKDNWP.WAVNCEHEKHVYSHC...KYEDYVIRMKEY E9G263_DAPPU/1-61 ..TLEEMESARIPLDKRDYCVDYLMKYLGCRNEKYP.WVYRCHHEKHEYLHC...QYEDYLIRMKEF T1HHP5_RHOPR/42-104 IATEEEMYAAKIPLKNRDYCAHYLLKFQKCRKDKFP.WVYRCHHEKHEYLHC...LYEEYLDRMKDF B7PUG2_IXOSC/47-109 VATEAELESARIPPKSRNYCAHKLIEFKACRKDHFP.LVYRCQHEKHEYDDC...LYQDYLIRYKEY A0A212F1I6_DANPL/39-101 IAKEEDLISARIPPKYRDYCAHHLLEYQVCRYKNVP.LMYKCAHEKHAYLNC...EHQDYVMRMKEF W5J798_ANODA/40-102 IATQEEMEAAKLPLEARDYCAHKLIQYRACRSDVWP.WAYKCAHEKHEYLNC...EYEDYILRLKEY Q29II4_DROPS/38-100 IATQEEMESVKLPLEDRDYCAHKLIAYQACRSDKFP.FVYQCAHEKHEYLTC...EYEDYVLRMKEF F7BC32_CIOIN/36-98 VATSEEMDKAALRREERDYCAHMFMDIMKCRREWFP.ELYNCRPLIEKYQHC...TVNDIKHRMLEY G4LVB0_SCHMA/39-101 ILTEEEMIAAGLKPYERDYCAHLLLAFRKCGDEHVI.PALYCSDLKHKYRHC...KESDLLHRMKEY F7G4X2_MONDO/40-102 VATQQEMNDAQLMLKHRDYCAHHLIRLLKCKRDTFP.NFLACGHEKHVWDLC...QHEDYVGRMKEF G3HD23_CRIGR/40-102 VATQQEMNNAQLTLQQRDYCAHYLIRLLKCKRDSFP.NFLACKHERHDWDYC...EHMDYVKRMKEF M4ACV7_XIPMA/40-102 IATQDQMNLAQLPLGQRDYCAHHLIKLMKCKRDNWP.NFLACKHERHDWDYC...EHQDYVMRMKEY A4II52_XENTR/35-97 VASQEQMNLAQVPLEQRDYCAHHLIELMKCKRDMWP.NFLACKHERHEWDLC...QHEDYVQRMKQY Q6P6E5_DANRE/40-102 VATQEQMNLAMLPVDQRDYCAHHLIKLMKCRRDMFP.NYMACDHQKHDWEYC...QHQDYVMRMKEY E5STE0_TRISP/43-106 IATDEEMDAWQLEYRDRDYCAHFYINHRRCLDNNRPFAYWNCKHERHELTKC...EWEDMVMRVKEF E0VEI7_PEDHC/681-743 HITEEEMDALKIPPEERDYCTHFLRDYYDCIYNKRP.LNFLCNREKHAYKHC...RQEDFYLQMKEF A0A2H2IDU2_CAEJA/43-106 KATWEEMEQWRLKPAQRDYCAHHLISLMKCQTQNAPFAGHACDGERGAWDKC...EYDDYIMRIKEF G8B8H3_CANPC/13-71 IPSQQELDDHNVPFYYRDKCASNLIEYYKCLDKGTS....FCNKTKDEFYKC...QYYLLKGRLDS. B5RTW7_DEBHA/10-72 .LSEEDLQKYKVPLRWRDRCAANFALYHICLKRQSANSSVDCKHDKHVWEEC...ENLDFIRRQKEL G3AFU7_SPAPN/10-72 .LSQDDMQKYNVPLKWRDRCAAYYALYNTCLVRERNSSKIDCKHDKHAWEEC...EFLDLQRRKQEL G3BDT0_CANTC/11-71 ..SDDDLQKYKVPLKWRDRCAFYFALYQTCLIRQSANGSVDCGHDKHAWEEC...ENLDLIRRKQE. F4P9U9_BATDJ/2-64 YITQEELNKTQIPLQWRDYCVHLLSELNQCRRESYF.APWKCVEEKLAWQKC...QYDDYHRRMRKL K0KJA2_WICCF/9-68 ...PSEMREAKIPLAWRDRCAALWIPLEKCRRETYF.LPWKCTQLRHDYEQC...QYLDFQRRVKEM B5FVG1_YARLI/8-69 .LSQEDMKKHKILLAYRDRCAALLVPLNECRKKNYY.MPWACGHERHEYEMC...EVADFQRRVKAM #=GR B5FVG1_YARLI/8-69 SS .--HHHHHHHT--TTT-STTGGGHHHHHHHHHHTTT.-TTSSHHHHHHHHHH...HHHHHHHHHHHH C4QWB4_KOMPG/37-99 IVSHEEMKQNKIPLAYRDRCARLLVPLNKCRKEGWY.MPWNCVQERHDYEQC...QYLDFKRRVKEL C1N513_MICPC/9-70 VATQKDMQDAKIDLAHRDFCAHLLIPLNECRKAEYY.IPWKCGHERHAYEKC...QYKEYMMRVAQ. A4RRY0_OSTLU/10-72 IASREDMKRAKLDIGYRDFCAHLLIPLNECRRKSFY.LPWKCGHERHVYEKC...QYKEYMKRVASV D7FZ56_ECTSI/11-72 KATEEEMVAAKMPMAFRDSCAHLLLPLNKCRRSTMY.LPWQCSHERHIYEKC...QYLDFKERKAM. Q6UKY7_CHLRE/11-72 KATQEQMDAADVPYHYRDYCAHMYIDYKECRLTSGFSWRTKCAHELHAYNKC...EYKEFKRRVA.. E1ZNP8_CHLVA/685-745 KATQEEMEAGNIDMASRDFCAHILLKLNKCRRDSLF.VPWKCEHERHAYELC...EYKDYKRRVA.. D3BII5_POLPP/18-75 IATREELEINNVPLNFRDYCAHLLIPLNKCRVKEYY.LPWKCGDERHAYEQC...QYDE....MKK. NDUB7_DICDI/19-77 IATQQQCEDKNLPLSFRDYCAHLLIPLNDCRVSTYY.APWKCMDEKHAYEGC...QYDEYLYR.... F4PPP8_CAVFA/20-81 IATSEELEANAIPLNFRDYCAHLLIPLNNCRVKESY.APWKCSDERHAYEKC...QYDEYLLRMKQ. #=GC SS_cons .--HHHHHHHT--TTT-STTGGGHHHHHHHHHHTTT.-TTSSHHHHHHHHHH...HHHHHHHHHHHH #=GC seq_cons hAopEEMppA+lPLtaRDhCAHhLIsLpcCRccpah.hsacCpcE+HsY-+C...pY-DahtRhKch //