Mitochondrial dysfunction is associated with neuroinflammation and neurodegenerative disease, but its role as a driver in these processes is uncertain. Understanding the pathogenesis of inherited mitochondrial disorders may help us to uncover mechanisms involved during acquired mitochondrial dysfunction. We review the mechanisms of mitochondrial dysfunction in Leber's hereditary optic neuropath...

Mitochondrial disorders were first described in 1962 (Luft, Ikkos, Palmieri, Ernster, & Afzelius, 1962), and were initially thought to be uniformly progressive and fatal with onset in infancy or early childhood, typically associated with severe neurological manifestations, including strokelike episodes, seizures, neuronal degeneration, organ failure and dementia. More recently, clinical observa...

Mitochondrial dysfunction mainly affects organs with high metabolic demand, primarily the brain. Epilepsy is a common phenotypic sign of both syndromic and non-syndromic mitochondrial diseases. in diseases poorly treatable often an unfavorable prognostic sign. Patients epilepsy are prescribed various anti-seizure medications. Mitochondriotoxic drugs should be avoided. Descriptions MELAS-syndrom...

OBJECTIVE To investigate the role of myeloid differentiation factor 88 (MyD88) on mitochondrial dysfunction of peritoneal leukocytes during polymicrobial sepsis. MATERIAL AND METHODS Polymicrobial peritonitis, a clinically relevant mouse model of sepsis, was generated by cecum ligation and puncture (CLP) in both male C57BL/6J wild-type (WT) and MyD88 knockout (MyD88(-/-)) mice. Twenty-four ho...

Podocyte injury underlies many forms of glomerular diseases. Our previous study showed that hyperoside, a naturally occurring flavonoid, could decrease albuminuria at the early stage of diabetic nephropathy by ameliorating renal damage and podocyte injury. However, its protective mechanism against podocyte injury is unknown. A previous study demonstrated that hyperoside might inhibit amyloid β-...

Mitochondrial dysfunction has gained recognition as a contributing factor in many diseases. The kidney is a kind of organ with high energy demand, rich in mitochondria. As such, mitochondrial dysfunction in the kidney plays a critical role in the pathogenesis of kidney diseases. Despite the recognized importance mitochondria play in the pathogenesis of the diseases, there is limited understandi...