8ca5

Cryo-EM structure NDUFS4 knockout complex I from Mus musculus heart (Class 3).

spinqualitylabelsall models 8ca5, resolution 3.90Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 8ca5 is a 10 chain structure with sequence from Mus musculus. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Method: Electron Microscopy, Resolution 3.9Å Ligands: 2MR, 3PE, CDL, EHZ, FES, FME, FMN, GTP, LMT, NDP, PC1, UQ9 Resources: FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT Function NDUF2_MOUSE Acts as a molecular chaperone for mitochondrial complex I assembly. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.[UniProtKB:Q8N183] Publication Abstract from PubMed Respiratory complex I (NADH:ubiquinone oxidoreductase) is essential for cellular energy production and NAD(+) 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(-/-) mouse, to define the structural, biochemical, and functional consequences of the absence of subunit NDUFS4. Cryo-EM analyses of the complex I from ndufs4(-/-) mouse hearts revealed a loose association of the NADH-dehydrogenase module, and discrete classes containing either assembly factor NDUFAF2 or subunit NDUFS6. Subunit NDUFA12, which replaces its paralogue NDUFAF2 in mature complex I, is absent from all classes, compounding the deletion of NDUFS4 and preventing maturation of an NDUFS4-free enzyme. We propose that NDUFAF2 recruits the NADH-dehydrogenase module during assembly of the complex. Taken together, the findings provide new molecular-level understanding of the ndufs4(-/-) mouse model and complex I-linked mitochondrial disease. Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4(-/-) mouse.,Yin Z, Agip AA, Bridges HR, Hirst J EMBO J. 2024 Jan;43(2):225-249. doi: 10.1038/s44318-023-00001-4. Epub 2024 Jan 2. PMID:38177503 From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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