Dataset Information
Cryo-EM structure NDUFS4 knockout complex I from Mus musculus heart
Ontology highlight
ABSTRACT:
SUBMITTER: Zhan Yin
PROVIDER: EMPIAR-11356 | biostudies-other |
REPOSITORIES: biostudies-other
Publications
Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4<sup>-/-</sup> mouse.
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]