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Details on Person A complex I intermediate of 315kDa (reestimated from the ori...
| Class:Id | Summation:6799201 |
|---|---|
| _displayName | A complex I intermediate of 315kDa (reestimated from the ori... |
| _timestamp | 2024-04-02 08:51:39 |
| created | [InstanceEdit:6799182] Jassal, Bijay, 2015-09-22 |
| modified | [InstanceEdit:9906777] Stephan, Ralf, 2024-04-02 [InstanceEdit:9906781] Stephan, Ralf, 2024-04-02 |
| text | A complex I intermediate of 315kDa (reestimated from the original 400kDa) is formed centred around the core subunits NADH dehydrogenase [ubiquinone] iron-sulfur proteins 2 and 3 (NDUFS2 and NDUFS3) with other complex I subunits and assembly factor subunits (forming IP and HP subcomplexes). The IP subcomplex is anchored to the inner mitochondrial membrane by NADH-ubiquinone oxidoreductase chain 1 (MT-ND1) (together with NDUFAF5 and/or 6) (Mckenzie & Ryan 2010, Andrews et al. 2013). NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 (NDUFAF8) and Protein preY (PYURF, NDUFAFQ) are required for stabilization of NDUFAF5 (Floyd et al., 2016; Rensvold et al., 2022; Peker et al., 2023). Defects in NDUFAF8 cause Complex I deficiency, manifesting as Leigh syndrome (Alston et al., 2019). |
| (summation) | [Reaction:6799191] Intermediate 2 binds MT-ND1:NDUFAF5:NDUFAF6 to form a 315kDa subcomplex [Homo sapiens] |
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