Mitochondria are small cellular constituents that generate cellular energy (ATP) by oxidative
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …

Symmetric, progressive, necrotizing lesions in the brainstem are a defining feature of Leigh
syndrome (LS). A mechanistic understanding of the pathogenesis of these lesions has been …

Mitochondrial dysfunction caused by aberrant Complex I assembly and reduced activity of
the electron transport chain is pathogenic in many genetic and age-related diseases. Mice …

Proteins embedded in the inner mitochondrial membrane (IMM) perform essential cellular
functions. Maintaining the folding state of these proteins is therefore of the utmost …

Mitochondrial deficits in energy production cause untreatable and fatal pathologies known
as mitochondrial disease (MD). Central nervous system affectation is critical in Leigh …

Leigh Syndrome (LS) is a mitochondrial disorder defined by progressive focal
neurodegenerative lesions in specific regions of the brain. Defects in NDUFS4, a subunit of …

Mitochondria and lysosomes are two organelles that carry out both signaling and metabolic
roles in cells. Recent evidence has shown that mitochondria and lysosomes are dependent …

Mitochondria are organelles that provide energy to cells through ATP production.
Mitochondrial dysfunction has long been postulated to mediate cellular declines that drive …

Background The vast majority of mitochondrial disorders have limited the clinical
management to palliative care. Rapamycin has emerged as a potential therapeutic drug for …

Mitochondrial diseases represent a spectrum of disorders caused by impaired mitochondrial
function, ranging in severity from mortality during infancy to progressive adult-onset disease …