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Translocase of inner mitochondrial membrane 21NADH:ubiquinone oxidoreductase complex assembly factor 8NADH:ubiquinone oxidoreductase subunit B10ECSIT signaling integratorNADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13NADH:ubiquinone oxidoreductase complex assembly factor 2NADH:ubiquinone oxidoreductase subunit B9NADH:ubiquinone oxidoreductase core subunit S2NADH:ubiquinone oxidoreductase core subunit S1NADH:ubiquinone oxidoreductase subunit A9FAD dependent oxidoreductase domain containing 1Acyl-CoA dehydrogenase family member 9BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperoneTranslocase of inner mitochondrial membrane domain containing 1NADH:ubiquinone oxidoreductase subunit AB1Distal membrane arm assembly complex 2Apoptosis inducing factor mitochondria associated 1NADH:ubiquinone oxidoreductase complex assembly factor 1NADH dehydrogenase, subunit 4 (complex I)NADH dehydrogenase, subunit 6 (complex I)NADH dehydrogenase, subunit 1 (complex I)NADH dehydrogenase, subunit 5 (complex I)MTND2NADH dehydrogenase, subunit 3 (complex I)Complex I intermediate-associated protein 30, mitochondrialNADH:ubiquinone oxidoreductase complex assembly factor 3NADH:ubiquinone oxidoreductase core subunit S8NADH:ubiquinone oxidoreductase subunit S4NADH:ubiquinone oxidoreductase subunit S5NADH:ubiquinone oxidoreductase core subunit S7NADH:ubiquinone oxidoreductase core subunit S3NADH:ubiquinone oxidoreductase core subunit V2NADH:ubiquinone oxidoreductase core subunit V1NADH:ubiquinone oxidoreductase subunit V3Transmembrane protein 126BOXA1L mitochondrial inner membrane proteinNADH:ubiquinone oxidoreductase subunit B5NADH:ubiquinone oxidoreductase subunit C1NADH:ubiquinone oxidoreductase subunit A2NADH:ubiquinone oxidoreductase subunit B6
P682
Mutations in the gene encoding C8orf38 block complex I assembly by inhibiting production of the mitochondria-encoded subunit ND1Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patientsAcyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex IC6ORF66 is an assembly factor of mitochondrial complex IMidA is a putative methyltransferase that is required for mitochondrial complex I functionMutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial diseaseThe arginine methyltransferase NDUFAF7 is essential for complex I assembly and early vertebrate embryogenesisNDUFAF5 Hydroxylates NDUFS7 at an Early Stage in the Assembly of Human Complex IMutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.Accessory subunits are integral for assembly and function of human mitochondrial complex I.Human mitochondrial complex I assembly is mediated by NDUFAF1.Mutations in Complex I Assembly Factor TMEM126B Result in Muscle Weakness and Isolated Complex I DeficiencyBi-allelic Mutations in NDUFA6 Establish Its Role in Early-Onset Isolated Mitochondrial Complex I Deficiency
P921
Q21100878-9F655499-CBD1-429B-AF7B-AA73541F0C4CQ21100878-F8541CF5-FE1E-4A37-8C60-AF91FB7976A1Q21100890-10E427D0-2E7B-4A9B-A95A-165C147255DDQ21100890-91B2FB74-4F80-4B28-9CBB-009374A87180Q21101248-C8E3988E-082D-4A61-B7FB-155BDA17E8DAQ21101884-0BD9B7D0-DB2A-4AA5-9E29-0023E19E306CQ21103360-FC43A2E1-7734-4502-AFD7-F9608575DB8CQ21104673-F62FA599-D727-4DBF-91B1-10E4D9F21C80Q21107983-A58A1692-D935-49A6-9477-ED5CBA0F3D13Q21109053-F2A4EB4A-C71B-4104-AAE5-95E0596674D1Q21109055-6CCC2D91-ECD9-40EE-A7BF-A8D050F2C54DQ21111709-F93E86D9-6AC4-4C13-A263-B7EC722760CDQ21112457-4C133743-4B2D-4894-B5C1-FE74A8197E04Q21112457-C43D8AFF-9CF0-4151-9B7A-6C0D19A354AEQ21113720-A17A90F5-5E82-435B-8CEB-D18E5848C412Q21113912-7506421E-F19D-4E2C-8D29-B2104BF1991DQ21114485-E0A2062D-EDD0-49C3-BDBC-C08B4BD3AF66Q21115295-CDEBD7F2-7C0E-4F70-96BF-8321697D3756Q21115362-356A264E-F9AB-485C-A5BA-39FD6A3ED710Q21115362-88F1BDDB-C5F5-4314-846A-0EA3FDC36601Q21116445-A75BCFED-466F-4077-BEF3-133D87076F30Q21117228-2B1B5B78-1029-4D6F-A9F8-149233162B81Q21117228-692F2FD7-866C-4FA0-AC05-C276464C019DQ21117228-790E8D39-7D98-4437-ACD2-A29125C3C1AEQ21117228-7ECC615E-79F2-477E-8F38-3A5CE133CAF8Q21117530-E050E09A-7959-4B5C-8498-A9077B0DCA39Q21117535-9706E572-84E9-4F92-BD12-0AC61DC2CE2AQ21117538-44CB5E62-7E55-4A7B-9C29-1FC273F8E554Q21117542-3B661184-C05C-4CB5-B65D-5ECC2FF8569BQ21117546-90A0E4B7-9176-48DB-818F-BF047BAB3EA9Q21117547-25C0E275-C0BC-41FD-BE40-6323C2FCEE48Q21117788-39750146-D3A3-4DD8-BED5-6BBC6ABA6269Q21118335-5CF8E6A3-F03B-43AD-850C-E8066572DE84Q21118335-9D31E4F7-06CE-474C-96C2-50B4E2E4E9ECQ21120099-9310FAEA-ED19-4307-90E1-6DBC58E150D1Q21120099-CFE637C9-68DF-4DD0-994F-C3E90945BEC8Q21120100-875EFCD8-E545-4F4C-964B-DE677CCAAAD3Q21120101-89478B56-FF4A-4102-B0B5-4FF37139DE5AQ21120101-C8CB096C-904E-4D0C-8354-6A67F2EAE1DAQ21120102-5CD7EA0D-80C1-4C9B-9E13-D0924F2B33B0
P682
Q24294279-0855669B-788F-42AF-9EEF-F9222B477AADQ24297605-0993366D-F16F-449F-BB6F-5DBDE479FD98Q24298522-F0FC10D8-9696-434F-AC72-F6E7C95D6366Q24305552-7C0FDC1C-BA43-488F-9CDD-B0DD79D96C87Q24307440-3E65F700-ACB3-4D72-A51E-9D77C7747233Q24336408-344C1203-22BC-4EEC-8C38-F67FC2CCBBB2Q28115372-D116FA9C-553C-41DB-8D66-251EA78D57DCQ28117068-8210A9A6-88C7-494F-856E-F881662B0AC9Q32884383-73AFA84E-55FE-4320-8967-740A6D3D500EQ32884468-712BB8F5-FB15-411C-A409-0D43EB116A78Q34458841-1B0EE983-CFC0-47E9-91CB-77A28B19BC95Q37218541-DD0B0903-7611-4D57-84C5-858AF3F9E8F6Q57023932-38A720F5-7EF1-456B-A278-CB75300434D2
P921
description
The aggregation, arrangement a ...... l respiratory chain complex I.
@en
biologisch proces
@nl
name
mitochondrial respiratory chain complex I assembly
@en
type
label
mitochondrial respiratory chain complex I assembly
@en
altLabel
GO:0032981
@en
mitochondrial NADH dehydrogenase complex (ubiquinone) assembly
@en
mitochondrial complex I assembly
@en
prefLabel
mitochondrial respiratory chain complex I assembly
@en
P2888
P686
GO:0032981