Knapp-Wilson, Amber published the artcileMaintenance of complex I and its supercomplexes by NDUF-11 is essential for mitochondrial structure, function and health in collection: mitochondria Amber Knapp-Wilson, SDS of cas: 97-67-6, the main research area is complexI NDUF11 mitochondrial structure health collection; Caenorhabditis elegans ; Cryo-electron tomography; Electron transfer chain; Mitochondria; Mitochondrial ultrastructure; NDUF-11; Respirasome; Respiration; Supercomplexes; Worm.
Mitochondrial supercomplexes form around a conserved core of monomeric complex I and dimeric complex III; wherein a subunit of the former, NDUFA11, is conspicuously situated at the interface. We identified nduf-11 (B0491.5) as encoding the Caenorhabditis elegans homolog of NDUFA11. Animals homozygous for a CRISPR-Cas9-generated knockout allele of nduf-11 arrested at the second larval (L2) development stage. Reducing (but not eliminating) expression using RNAi allowed development to adulthood, enabling characterization of the consequences: destabilization of complex I and its supercomplexes and perturbation of respiratory function. The loss of NADH dehydrogenase activity was compensated by enhanced complex II activity, with the potential for detrimental reactive oxygen species (ROS) production Cryo-electron tomog. highlighted aberrant morphol. of cristae and widening of both cristae junctions and the intermembrane space. The requirement of NDUF-11 for balanced respiration, mitochondrial morphol. and development presumably arises due to its involvement in complex I and supercomplex maintenance. This highlights the importance of respiratory complex integrity for health and the potential for its perturbation to cause mitochondrial disease.
Journal of Cell Science published new progress about Amino acids Role: BSU (Biological Study, Unclassified), BIOL (Biological Study). 97-67-6 belongs to class alcohols-buliding-blocks, name is (S)-2-hydroxysuccinic acid, and the molecular formula is C4H6O5, SDS of cas: 97-67-6.
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