DIPHOSPHATIDYL GLYCEROL; BIS-(1,2-DIACYL-SN-GLYCERO-3-PHOSPHO)-1',3'-SN-GLYCEROL

NADPH DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE PHOSPHATE

S-[2-({N-[(2S)-2-hydroxy-3,3-dimethyl-4-(phosphonooxy)butanoyl]-beta-alanyl}amino)ethyl] tetradecanethioate

Atomic structure of the entire mammalian mitochondrial complex I.

For the fourteen core subunits we used as a starting point our previous structure from T. thermophilus (PDB 4HEA) with sequence adjusted to ovine and side-chains rebuilt in SQWRL4. These are chains 1, 2, 3, 4, 5, 6, 9, H, A, J, K, N, M and L (with the same chain IDs as in 4HEA).

For the remaining supernumerary subunits it was mostly de novo building with some help from homology modeling, which in most cases was not very useful due to low sequence homology. In few cases models produced by Phyre2 server were useful and were used as a starting point, due to the presence of templates with high coverage and confidence:

42kDa (chain k) - PDB ID 1P6X (xray)
39kDa (chain d) - PDB ID 3WJ7 (xray)
SDAPs (chains j and X) - PDB ID 2DNW (NMR)
B8 (chain e) - PDB ID 1S3A (NMR)

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial

Cell death regulatory protein GRIM-19,Complex I-B16.6,CI-B16.6,Gene associated with retinoic-interferon-induced mortality 19 protein,GRIM-19,NADH-ubiquinone oxidoreductase B16.6 subunit

Complex I-ASHI,NADH-ubiquinone oxidoreductase ASHI subunit

Complex I-KFYI,CI-KFYI,NADH-ubiquinone oxidoreductase KFYI subunit

FSGDTTAPKKTSFGSLKDEDRIFTNLYGRHDWRLKGAQSRGDWYKTKEILLKGPDWILGEVKTSGLRGRGGAGFPTGLKW
SFMNKPSDGRPKYLVVNADEGEPGTCKDREIIRHDPHKLVEGCLVGGRAMGARAAYIYIRGEFYNEASNLQVAIREAYEA
GLIGKNACGSGYDFDVFVVRGAGAYICGEETALIESIEGKQGKPRLKPPFPADVGVFGCPTTVANVETVAVSPTICRRGG
AWFASFGRERNSGTKLFNISGHVNNPCTVEEEMSVPLKELIEKHAGGVTGGWDNLLAVIPGGSSTPLIPKSVCETVLMDF
DALIQAQTGLGTAAVIVMDRSTDIVKAIARLIEFYKHESCGQCTPCREGVDWMNKVMARFVRGDARPAEIDSLWEISKQI
EGHTICALGDGAAWPVQGLIRHFRPELEERMQRFAQQHQARQAAS

GAGGALFVHRDTPKNNPETPFDFTPENYKRIEAIVKNYPEGHKAAAVLPVLDLAQRQNGWLPISAMNKVAEILQVPPMRV
YEVATFYTMYNRKPVGKYHIQVCTTTPCMLRNSDSILEAIQKKLGIKVGETTPDKLFTLIEVECLGACVNAPMVQINDNY
YEDLTPKDIEEIIDELKAGKIPKPGPRSGRFSCEPAGGLTSLTEPPKGPGFGVQAGL

TATAASNLIEVFVDGQSVMVEPGTTVLQACEKVGMQIPRFCYHERLSVAGNCRMCLVEIEKAPKVVAACAMPVMKGWNIL
TNSEKTKKAREGVMEFLLANHPLDCPICDQGGECDLQDQSMMFGSDRSRFLEGKRAVEDKNIGPLVKTIMTRCIQCTRCI
RFASEIAGVDDLGTTGRGNDMQVGTYIEKMFMSELSGNIIDICPVGALTSKPYAFTARPWETRKTESIDVMDAVGSNIVV
STRTGEVMRILPRMHEDINEEWISDKTRFAYDGLKRQRLTEPMVRNEKGLLTHTTWEDALSRVAGMLQSCQGNDVAAIAG
GLVDAEALIALKDLLNRVDSDTLCTEEVFPTAGAGTDLRSNYLLNTTIAGVEEADVVLLVGTNPRFEAPLFNARIRKSWL
HNDLKVALIGSPVDLTYRYDHLGDSPKILQDIASGSHPFSQVLQEAKKPMVVLGSSALQRNDGAAILAAVSNIAQKIRTS
SGVTGDWKVMNILHRIASQVAALDLGYKPGVEAIRKNPPKMLFLLGADGGCVTRQDLPKDCFIVYQGHHGDVGAPIADVI
LPGAAYTEKSATYVNTEGRAQQTKVAVMPPGLAREDWKIIRALSEIAGMTLPYDTLDQVRNRLEEVSPNLVRYDDVEGAN
YFQQASELSKLVNQQLLADPLVPPQLTIKDFYMTDSISRASQTMAKCVKAVTEGAHAVEEPSIC

MYPTKETAHWKPPPWNDVDPPKDTLVSNLTLNFGPQHPAAHGVLRLVMELSGEMVRKCDPHIGLLHRGTEKLIEYKTYLQ
ALPYFDRLDYVSMMCNEQAYSLAVEKLLNIQPPPRAQWIRVLFGEITRLLNHIMAVTTHALDIGAMTPFFWMFEEREKMF
EFYERVSGARMHAAYVRPGGVHQDLPLGLMDDIYEFSKNFSLRIDELEEMLTNNRIWRNRTVDIGVVTAEDALNYGFSGV
MLRGSGIQWDLRKTQPYDVYDQVEFDVPIGSRGDCYDRYLCRVEEMRQSIRIISQCLNKMPPGEIKVDDAKVSPPKRAEM
KTSMESLIHHFKLYTEGYQVPPGATYTAIEAPKGEFGVYLVSDGSSRPYRCKIKAPGFAHLAGLDKMSKGHMLADVVAII
GTQDIVFGEVDR

ESASADTRPTVRPRNDVAHKQLSAFGEYVAEILPKYVQQVQVSCFSELEICIHPDGVIPVLTFLRDHSNAQFKSLADLTA
VDIPTRQNRFEIVYNLLSLRFNSRIRVKTYTDELTPVESSVSVYKAANWYEREIWDMFGVFFANHPDLRRILTDYGFEGH
PFRKDFPLSGYVELRYDDEVKRVVAEPVELAQEFRKFDLNSPWEAFPAYRQPPESLKLEAGDKKPEAK

PSSTQPAVSQARAVVPKPAALPSSRGEYVVAKLDDLINWARRSSLWPMTFGLACCAVEMMHMAAPRYDMDRFGVVFRASP
RQSDVMIVAGTLTNKMAPALRKVYDQMPEPRYVVSMGSCANGGGYYHYSYSVVRGCDRIVPVDIYVPGCPPTAEALLYGI
LQLQKKIKREKRLRIWYRR

TYKYVNLREPSMDMKSVTDRAAQTLLWTELIRGLGMTLSYLFREPATINYPFEKGPLSPRFRGEHALRRYPSGEERCIAC
KLCEAVCPAQAITIEAEPRADGSRRTTRYDIDMTKCIYCGFCQEACPVDAIVEGPNFEFSTETHEELLYNKEKLLNNGDK
WEAEIAANIQADYLYR

MFMINVLTLIIPILLAVAFLTLVERKVLGYMQFRKGPNVVGPYGLLQPIADAIKLFIKEPLRPATSSISMFILAPILALT
LALTMWIPLPMPYPLINMNLGVLFMLAMSSLAVYSILWSGWASNSKYALIGALRAVAQTISYEVTLAIILLSVLLMNGSF
TLSTLIITQEQVWLIFPAWPLAMMWFISTLAETNRAPFDLTEGESELVSGFNVEYAAGPFALFFMAEYANIIMMNIFTTT
LFLGAFHNPYMPELYTINFTIKSLLLSITFLWIRASYPRFRYDQLMHLLWKNFLPLTLALCMWHVSLPILLSSIPPQT

MNPIILIIILMTVMLGTIIVMISTHWLLIWIGFEMNMLAIIPIMMKKHNPRATEASTKYFLTQSTASMLLMMAIIINLMF
SGQWTVMKLFNPMASMLMTMALAMKLGMAPFHFWVPEVTQGIPLSSGLILLTWQKLAPMSVLYQILPSINLDLILTLSIL
SITIGGWGGLNQTQLRKIMAYSSIAHMGWMTAVLLYNPTMTLLNLIIYIIMTSTMFTLFMANSTTTTLSLSHTWNKAPIM
TILVLITLLSMGGLPPLSGFMPKWMIIQEMTKNDSIILPTLMAITALLNLYFYMRLTYSTALTMFPSTNNMKMKWQFPTT
KRMTLLPTMTVLSTMLLPLTPILSILE

MNLMITLLTNFTLATLLVTIAFWLPQLNVYSEKTSPYECGFDPMGSARLPFSMKFFLVAITFLLFDLEIALLLPLPWASQ
TTNLNTMLTMALLLIFLLAVSLAYEWTQKGLEWTE

MLKYIIPTMMLMPLTWLSKNSMIWINTTLHSLLISLTSLLLLNQFGDNSLNFSLTFFSDSLSTPLLILTMWLLPLMLMAS
QHHLSKENLARKKLFISMLILLQLFLIMTFTATELIFFYIMFEATLVPTLIIITRWGNQTERLNAGLYFLFYTLAGSLPL
LVALIYIQNTMGSLNFLILQYWVQPMPNSWSNTFMWLACMMAFMVKMPLYGLHLWLPKAHVEAPIAGSMVLAAILLKLGG
YGMMRITLLLNPITDFMAYPFIMLSLWGMIMTSSICLRQTDLKSLIAYSSVSHMALVIVAILIQTPWSYMGATALMIAHG
LTSSMLFCLANSNYERVHSRTMILARGLQTLLPLMAAWWLLASLTNLALPPSINLIGELFVVMSTFSWSNITIILMGLNM
VITALYSLYMLITTQRGKHTHHINNILPSFTRENALMSLHMLPLLLLSLNPKIILGPLY

MSLVYMNIMMAFTVSLTGLLMYRSHLMSSLLCLEGMMLSLFILATLMILNSHFTLASMMPIILLVFAACEAALGLSLLVM
VSNTYGTDYVQNLNLLQC

MNLFSSLTLVTLILLTMPIAAINFNTHKFTNYPLYVKTTISCAFITSMIPTMMFIHTGQEMIISNWHWLTIQTLKLSLSF
KMDFFSMMFVPVALFVTWSIMEFSMWYMHSDPNINQFFKYLLLFLITMLILVTANNLFQLFIGWEGVGIMSFLLIGWWYG
RTDANTAALQAILYNRIGDIGFILAMAWFLINLNTWDLQQIFMLNPNDSNLPLMGLILAATGKSAQFGLHPWLPSAMEGP
TPVSALLHSSTMVVAGIFLLIRFYPLTENNKFGQSIMLCLGAMTTLFTAMCALTQNDIKKIIAFSTSSQLGLMMVTIGIN
QPHLAFLHICTHAFFKAMLFMCSGSIIHSLNDEQDIRKMGGLFKAMPFTTTALIIGSLALTGMPFLTGFYSKDLIIESAN
TSYTNAWALLMTLVATSFTAIYSTRIIFFALLGQPRFPTLININENNPFLINSIKRLLIGSLFAGFIISNNIPPMTIPQM
TMPHYLKMTALTVTILGFILALEISNTTHYLKFNYPSNTFKFSNLLGYYPTIMHRLTPYMNLTMSQKSASSLLDLIWLET
ILPKTISLAQMKMSTTITSQKGLIKLYFLSFLITILIST

MMTYIVFILSIIFVMGFVGFSSKPSPIYGGLGLIVSGGVGCGIVLNFGGSFLGLMVFLIYLGGMMVVFGYTTAMATEQYP
EVWVSNKVVLGTFITGLLMEFLMVYYVLKDKEVEIVFKFNGMGDWVIYDTGDSGFFSEEAMGIAALYSYGTWLVIVTGWS
LLIGVVVIMEITRGN

GVRTSPTGEKVTHTGQVYDDEDYRRVRFVGRQKEVNENFAIDLIAEQPVSQVGSRVISCDGGGGALGHPRVYINLDKETK
TGTCGYCGLQFRQQHH

AQDQTRDTQLITVDEKLDITTLTGVPEEHIKTRKARIFVPARNNMQSGVNNTKKWKMEFDTRERWENPLMGWASTADPLS
NLVLTFSTKEDAIAFAEKNGWSYDVEERKVPKPKSKSYGANFSWNKRTRVSTK

LHHAVIPHGKGGRSSVSGIVATVFGATGFLGRYLVNHLGRMGSQVIVPYRCEPYDTMHLRPMGDLGQIIFMDWNGRDKDS
IRRAVEHSNVVINLVGREWETKNFDFEDVFVKIPQAIAQVSKEAGVEKFIHISHLNADIKSSSKYLRNKAVGEKEVRETF
PEATIIKPADIFGREDRFLNYFANIRWFGGVPLISLGKKTVKQPVYIVDVTKGIINAIKDPDARGKTFAFVGPNRYLLFD
LVQYVFAVAHRPFLPYPMPHFAYRWIGRLFEISPFEPWTTRDKVERIHTTDRTLPHLPGLEDLGVQATPLELKAIEVLRR
HRTYRWLSSEIEDVQPAK

AAAAAIRGVRGKLGLREIRIHLCQRSPGSQGVRDFIEKRYVELKKANPDLPILIRECSDVQPKLWARYAFGQEKNVSLNN
FSADQVTRALENVLSSKA

AGLLKKTTGLVGLAVCETPHERLKILYTKILDVLGHIPKNAAYRKYTEQITNEKLSIVKAEPDVKKLEEQLQGGQIEEVI
LQAENELSLARKMIQWKPWEPLVEEPPASQWKWPI

AASGLRQAAVAASTSVKPIFSRDMNEAKRRVRELYRAWYREVPNTVHLFQLDISVKQGRDKVREMFKKNAHVTDPRVVDL
LVIKGKMELEETINVWKQRTHVMRFFHETEAPRPKDFLSKFYVGHDP

ASATRLIQGLRNWASGRDLQAKLQLRYQEISKRTQPPPKLPVGPSHRLSNNYYCARDGRREAMPPSIVMSSQKVLVAGKP
AESSAVAASEKKAVSPAPPIKRWELSQDEPYL

MELLQVLKRGLQQVSGHGGLRGYLRVLFRANDVRVGTLVGEDKYGNKYYEDNKQFFGRHRWVIYTTEMNGKNTFWDVDGS
MVPPEWHRWLHCMTDDPPTVKPPTARKFIWTNHKFNLSGTPQQYVPYSTTRKKIQEWVPPSTPYK

SDAPPLTLEGIKDRVLYVLKLYDKIDPEKLSVNSHFMKDLGLDSLDQVEIIMAMEDEFGFEIPDIDAEKLMCPQEIVDYI
ADKKDVYE

LQYGPLAYILGEKTTKKMTENSKLITVDGNICSGKSKLAKEVAEKLGLKHFPEAGIHYADSTTGDGKPLPVRFSGNCSLE
KFYDDPKSNDGNSYRLQAWLYASRLLQYADALEHLLSTGQGVVLERSIYSDFVFLEAMYRQGFIRKQCVDHYNQVKKVTV
CEYLPPHVVIYVDVPVSEVQSRIQKKGNPHEMKITSAYLQDIENVYKGTFLPEMSEKCEVLQYSAWEAEDAEKVVEDIQY
LKYDKGPWLDQDDRKLHNLRMLVQDKLEVLNYTSIPVFLPEVTIGAHQSDRVFQEFTELPGRKYRAGYNEDVGDKWIWLK

PFFDVQKKLGVDLDHWMTIQSAEQPHRIPARCHAFEKEWIECAHGIGSIRAEKECKIEFEDFRECLLRQKTMKRLNAIKR
QRDKLIKEGKYTPPPHHSGQEDLRP

APRVAAFLKNVWAKEPVLVASFAIAGLAVILPTLSPYTKYSLMINRATPYNYPVPLRDDGNMPDVPSHPQDPQGPSLEWL
KRL

AHGHGHEHGPSKMELPDYKQWKIEGTPLETVQEKLAARGLRDPWGRNEAWRYMGGFANNVSFVGALLKGFKWGFAAFVVA
VGAEYYLESQKKDKKHH

MMTGRQARAPLQFLPDEARSLPPPKLTDPRLAYIGFLGYCSGLIDNAIRRRPVLSAGLHRQFLYITSFVFVGYYLLKRQD
YMYAVRDHDMFSYIKSHPEDFPEKDKKTYREVFEEFHPVR

SFPKYEPSRLASLPTTLDPAEYDISSETRKAQAERLAIRSRLKREYQLQYNDPSRRGVVEDPALIRWTCARSANVYPNFR
PNTKTSLLGALFGIGPLIFWYYVFKTDRDRKEKLIQEGKLDRTFNISY

AASKVKQDMPPVGGYGPIDYKRNLPRRGLSGYSMFAVGIGALLFGYWSMMRWNRERRRLQIEDFEARIALMPLLQAEKDR
RVLQMLRENLEEEATIMKDVPGWKVGESVFHTTRWVTPMMGELYGLRTGEEILSSTYGFIWYT

SGYTPDEKLRLQQLRELRRRWLKDQELSPREPVLPPRRVSPVERFWNKFLQDGALWKNVIYKTYRHSIFAFTHVLIPVWI
IHYYLKYHVTTKPYTIVEKKPRIFPGDTILETGEVIPPMKEFPDQHH

GAHLARRYLGDASVEPEPLRMPTFPPDYGFPERKEREMVATQQEMNDAQLVLQQRDYCAHYLIRFLKCKRDSFPNFLACK
HEQHDWDYCEHLDYVKRMKEFERERRLLQRKKRREQREADMAKGLGPGEVAPEVAL

AFLPSAAYLTHQQKVLQLYKRALRHLESRCVHRDKYRYFACLLRARFDEHKNEKDMVKATQLLREAEKEFWHGQHPQPYI
FPESPGGTSYERYECYKVPEWCLDDWHPSEKAMYPDYFAKREQWKKLRRESWEREVKQLQEETPVGGPRTEALPPARKQG
DLPPLWWHIVTRPRERPM

ASHITKDMLPGPYPKTPEERAAAAKKYNMRVEDYEPYPDDGMGYGDYPKLPDRSQQERDPWYDWDHPDLRLNWGEPMHWD
LDMYIRNRVDTSPTPVNWNLMCKHLFGFVAFMLFMFWVGETYPTYQPVGPKQYPYNNLYLERGGDPNKEPEPVVHYEI

ESSSSRAVIAPSTLAGKRPSEPTLRWQEDPEPEDENLYEKNPDSHGYDKDPAVDVWNMRVVFFFGFSIVLVLGSTFVAYL
PDYRMQEWARREAERLVKYREAHGLPLMESNCFDPSKIQLPEDED

PDSWDKDVYPEPPRRTPAPSPQTSLPNPITYLTKAFDLLVDRPVTLVREFIERQHAKNKYYYYHREFRRVPDITECHEKD
VLCMFEAEMQWKRDYKVDQEIVNIIQERLKACQQREGESHRQNCAKELQQFTQVVKAYQDRYHDLGAHYSARKCLAKQKQ
RMLAERKATKEAAAA

PGIVELPSLEDLKVQEVKVSSSVLKAAAHHYGAQCDKPNKEFMLCRWEEKDPRRCLEEGKLVNQCALEFFRQIKRHCAEP
FTEYWTCIDYSGLQLFRRCRKEQAQFDKCVLDKLGWVRPDLGELSKVTKVKTDRPLPENPYHSRARPEPNPEVEGDLKPA
RHGSRLFFWTM

SGDHGKRLFIIKPSGFYDKRFLKLLRFYILLTGIPVVIGITLINVFIGEAELAEIPEGYVPEHWEYFKHPISRWIARTFF
DAPEKNYERTMAILQIESEKAELRLKELEVRRLMRAKGDGPWFQYPTIDKALIDHSPKATPDN

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Medical Research Council (United Kingdom)
Map of the whole mammalian complex I used to create the Combined Map
Map of the peripheral arm of mammalian complex I used to create the Combined Map
Map of the membrane domain of mammalian complex I used to create the Combined Map
Mitochondrial complex I, 51 kDa subunit, Mitochondrial complex I, 24 kDa subunit, Mitochondrial complex I, 75 kDa subunit, Mitochondrial complex I, 49 kDa subunit, Mitochondrial complex I, 30 kDa subunit, Mitochondrial complex I, PSST subunit, Mitochondrial complex I, TYKY subunit, NADH-ubiquinone oxidoreductase chain 1 (E.C.1.6.5.3), NADH-ubiquinone oxidoreductase chain 2 (E.C.1.6.5.3), NADH-ubiquinone oxidoreductase chain 3 (E.C.1.6.5.3), NADH-ubiquinone oxidoreductase chain 4 (E.C.1.6.5.3), NADH-ubiquinone oxidoreductase chain 4L (E.C.1.6.5.3), NADH-ubiquinone oxidoreductase chain 5 (E.C.1.6.5.3), NADH-ubiquinone oxidoreductase chain 6 (E.C.1.6.5.3), Mitochondrial complex I, 10 kDa subunit, Mitochondrial complex I, 13 kDa subunit, Mitochondrial complex I, 18 kDa subunit, Mitochondrial complex I, 39 kDa subunit, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2, Mitochondrial complex I, B13 subunit, Uncharacterized protein, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12, Acyl carrier protein, mitochondrial, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3, NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13, NADH dehydrogenase (Ubiquinone) 1 beta, NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial, NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial, NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial, NADH dehydrogenase 1 alpha subcomplex, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8, Mitochondrial complex I, B14.7 subunit
NADH:ubiquinone, oxidoreductase, complex I, mammalian, mitochondrial