Dataset Information
Single particle cryo-EM dataset of Mus musculus mitochondrial complex I with ND6 P25L mutation
Ontology highlight
ABSTRACT:
SUBMITTER: Hannah Ruth Bridges
PROVIDER: EMPIAR-10605 | biostudies-other |
REPOSITORIES: biostudies-other
Publications
Structural basis for a complex I mutation that blocks pathological ROS production.
Nature communications 20210129 1
Mitochondrial complex I is central to the pathological reactive oxygen species (ROS) production that underlies cardiac ischemia-reperfusion (IR) injury. ND6-P25L mice are homoplasmic for a disease-causing mtDNA point mutation encoding the P25L substitution in the ND6 subunit of complex I. The cryo-EM structure of ND6-P25L complex I revealed subtle structural changes that facilitate rapid conversion to the "deactive" state, usually formed only after prolonged inactivity. Despite its tendency to a ...[more]