Mitochondrial complex I assembly model OXPHOS system (Homo sapiens)

From WikiPathways

Jump to: navigation, search
MT-ND5NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1DMAC1NDUFAF2179, 12, 17NDUFS31711, 17171711, 171713179, 12, 1721117, 25111427, 2811, 172011CHAPERONESTimeline of incorporationAdditional subunitsfor final complex formation+COMPLEX 1ND4-moduleComplex I assembly:EC 1.6.5.3IMSND2-moduleQ/ND1-moduleQ-moduleN-moduleDMAC1Electron Transport Chain PWMT-ND5[4Fe-4A]clustersNDUFA1NDUFB5FOXRED1MT-ND4NDUFA5TMEM186TMEM126BMT-ND3NDUFAF2COA1MT-ND1TIMMDC1NDUFV1NDUFA6NDUFA3NDUFS1NDUFAF39, 12, 17C11CC3, 22, 23, 2710, 1511, 18, 24CNDUFV217NDUFA2NDUFV1NDUFV2NDUFS1NDUFA2NDUFA7NDUFA12NDUFS4NDUFS6NDUFV3NDUFB10NDUFB11NDUFB6NDUFB5NDUFB10NDUFB11NDUFB6NDUFB1NDUFB4NDUFB5NDUFB10NDUFB11NDUFB6MT-ND4NDUFB1NDUFB4ATP5SL10, 17TMEM7011, 22, 26MTND2NDUFC1NDUFC2NDUFAF16, 19ECSIT29ACAD95, 21MT-ND3MT-ND6MT-ND4LMT-ND6MT-ND4LMTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9MT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9COA1C11, 18, 24COA118, 24CTMEM18611COA1CC18, 24NDUFA10NDUFS5MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS511, 17TMEM186COA12TMEM126BNDUFAF1ECSIT6, 19ACAD9295, 21NDUFAF616NUBPLNDUFS2NDUFAF7[4Fe-4A]clustersNUBPL27, 28[4Fe-4A]clustersNDUFS2MMNDUFS8NDUFAF58NDUFS7COOHNDUFS7NDUFAF44necessary to maintain normal MT-ND1 synthesisC[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS88NDUFS7NDUFAF616NDUFAF3NDUFAF4C4C11NDUFA8NDUFA13[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS88NDUFS7NDUFAF6NDUFAF316NDUFAF4C4MT-ND1CTIMMDC110, 15C[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS8NDUFS78NDUFAF616NDUFAF3NDUFAF4CC4MT-ND1TIMMDC110, 15CNDUFA3NDUFA8NDUFA13NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1ND5-moduleMT-ND5NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1DMAC117MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5COA111, 18, 24COA111, 18, 24TMEM18611COA118, 24TMEM18611COA1FOXRED118, 24ATP5SL3, 22, 23, 27TMEM7010, 1711, 22, 26TMEM186C11COA1C18, 24Mitochondrial Complex I Assembly (MCIA) complex2, 15MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5FOXRED1ATP5SL3, 22, 23, 2710, 17TMEM7011, 22, 26TMEM186COA1C11C18, 24[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS88NDUFS7NDUFAF616NDUFAF3CNDUFAF4MT-ND14CTIMMDC110, 15CNDUFA3NDUFA8NDUFA13MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5FOXRED13, 22, 23, 27ATP5SL10, 17TMEM70TMEM18611, 22, 26CCOA11118, 24C[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS8NDUFS78NDUFAF616NDUFAF3NDUFAF4C4MT-ND1CTIMMDC110, 15CNDUFA3NDUFA8NDUFA13MT-ND5NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1DMAC117NDUFAF29, 12, 17MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5FOXRED13, 22, 23, 27ATP5SLTMEM7010, 17TMEM18611, 22, 26COA111CC18, 24[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS88NDUFS7NDUFAF616NDUFAF3CNDUFAF44CMT-ND1TIMMDC1C10, 15NDUFA3NDUFA8NDUFA13NDUFA6NDUFA7NDUFA12NDUFS4NDUFS6NDUFV3NDUFV1NDUFV2NDUFS1NDUFA2IMMatrixTMEM126B