4 results found
    1. Cell Biology

    QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

    Virginia Guarani et al.
    Building on previous work (Guarani et al., 2015), MICOS (mitochondrial contact site) assembly and cristae junction formation are shown to have a critical role in human health.
    1. Neuroscience

    Defined neuronal populations drive fatal phenotype in a mouse model of Leigh syndrome

    Irene Bolea et al.
    Glutamatergic brainstem neurons drive motor and respiratory deficits, and GABAergic basal ganglia neurons cause hypothermia and fatal epileptic events, in a model of mitochondrial disease.
    1. Neuroscience

    Alleviation of neuronal energy deficiency by mTOR inhibition as a treatment for mitochondria-related neurodegeneration

    Xinde Zheng et al.
    Rapamycin treatment inhibits mTOR activity and preserves ATP levels in neurons derived from induced pluripotent stem cells from a maternally inherited Leigh syndrome patient.
    1. Biochemistry and Chemical Biology
    2. Genetics and Genomics

    The long non-coding RNA Cerox1 is a post transcriptional regulator of mitochondrial complex I catalytic activity

    Tamara M Sirey et al.
    Mitochondrial energy metabolism is post-transcriptionally regulated through an interplay between long and short non-coding RNAs.

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