(Q45317377)

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Complex I biogenesis

An instance of the biological pathway in Homo sapiens with Reactome ID (R-HSA-6799198)

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8 July 2022

mitochondrial respiratory chain complex I assembly

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Respiratory electron transport

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https://identifiers.org/reactome:R-HSA-6799198

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NUBPL transfers 4Fe-4S to Complex I subunits

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NUBPL transfers 4Fe-4S to NDUFV1, V2

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NDUF subunits bind to form the IP subcomplex

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IP subcomplex binds NDUFAF3, NDUFAF4, TIMMDC1 to form Intermediate 1

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Intermediate 1 binds HP subcomplex to form Intermediate 2

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Intermediate 2 binds MT-ND1:NDUFAF5:NDUFAF6 to form a 315kDa subcomplex

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COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6, NDUFB6 bind the MCIA complex to form a 370kDa subcomplex

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The 315kDa subcomplex binds the 370kDa subcomplex to form the 550kDa complex

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ND4, ND5 bind the 550kDa complex to form the 815kDa complex

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NDUF subunits bind to form the FP subcomplex

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Peripheral arm subunits bind the 815kDa complex to form a 980kDa complex

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The MCIA complex, NDUFAF2-7 all dissociate from the 980kDa complex, resulting in Complex I

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Assembly of mitochondrial cytochrome c-oxidase, a complicated and highly regulated cellular process

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Assembly factors for the membrane arm of human complex I.

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Assembly factors of human mitochondrial complex I and their defects in disease

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Understanding mitochondrial complex I assembly in health and disease

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Identifiers

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8 July 2022

(Q45317377)

Complex I biogenesis

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