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Cryo-EM structure NDUFS4 knockout complex I from Mus musculus kidney


ABSTRACT:

SUBMITTER: Zhan Yin 

PROVIDER: EMPIAR-11606 | biostudies-other |

REPOSITORIES: biostudies-other

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Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4<sup>-/-</sup> mouse.

Yin Zhan Z   Agip Ahmed-Noor A AA   Bridges Hannah R HR   Hirst Judy J  

The EMBO journal 20240102 2


Respiratory complex I (NADH:ubiquinone oxidoreductase) is essential for cellular energy production and NAD<sup>+</sup> homeostasis. Complex I mutations cause neuromuscular, mitochondrial diseases, such as Leigh Syndrome, but their molecular-level consequences remain poorly understood. Here, we use a popular complex I-linked mitochondrial disease model, the ndufs4<sup>-/-</sup> mouse, to define the structural, biochemical, and functional consequences of the absence of subunit NDUFS4. Cryo-EM anal  ...[more]

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