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Searching for alternative toxicology testing systems: The response of isolated mitochondria from Saccharomyces cerevisiae, potato tuber, and mouse liver to a toxic insult [PDF]

open access: yesTrends in Pharmaceutical Sciences, 2021
Mitochondria are cellular power plants known as essential organelles for energy (ATP) metabolism. However, today it is evident that various vital compounds are partially or exclusively synthesized in mitochondria. Moreover, this organelle plays a pivotal
Pouria Mobasher   +5 more
doaj   +1 more source

Echinochrome A Increases Mitochondrial Mass and Function by Modulating Mitochondrial Biogenesis Regulatory Genes

open access: yesMarine Drugs, 2014
Echinochrome A (Ech A) is a natural pigment from sea urchins that has been reported to have antioxidant properties and a cardio protective effect against ischemia reperfusion injury.
Seung Hun Jeong   +11 more
doaj   +1 more source

Recent advances in understanding the molecular genetic basis of mitochondrial disease

open access: yesJournal of Inherited Metabolic Disease, 2019
Mitochondrial disease is hugely diverse with respect to associated clinical presentations and underlying genetic causes, with pathogenic variants in over 300 disease genes currently described.
K. Thompson   +9 more
semanticscholar   +1 more source

Mitochondrial disease in flies

open access: yesBiochimica et Biophysica Acta (BBA) - Bioenergetics, 2004
The Drosophila mutant technical knockout (tko), affecting the mitochondrial protein synthetic apparatus, exhibits respiratory chain deficiency and a phenotype resembling various features of mitochondrial disease in humans (paralytic seizures, deafness, developmental retardation).
Kevin M.C. O'Dell   +5 more
openaire   +3 more sources

Neuropathology of mitochondrial diseases

open access: yesBioscience Reports, 2007
The term “mitochondrial diseases” (MD) refers to a group of disorders related to respiratory chain dysfunction. Clinical features are usually extremely heterogeneous because MD may involve several tissues with different degrees of severity. Muscle and brain are mostly affected, probably because of their high dependence on oxidative metabolism.
FILOSTO M   +7 more
openaire   +5 more sources

Oral delivery of carrier-free dual-drug nanocrystal self-assembled microspheres improved NAD+ bioavailability and attenuated cardiac ischemia/reperfusion injury in mice

open access: yesDrug Delivery, 2021
Nicotinamide riboside (NR), as a dietary supplement, can be converted to nicotinamide adenine dinucleotide (NAD+) in cells to support mitochondrial energy metabolism.
Hongfei Nie   +5 more
doaj   +1 more source

Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease

open access: yesScientific Reports, 2019
Leigh syndrome is a mitochondrial disease characterized by neurological disorders, metabolic abnormality and premature death. There is no cure for Leigh syndrome; therefore, new therapeutic targets are urgently needed. In Ndufs4-KO mice, a mouse model of
C. F. Lee   +5 more
semanticscholar   +1 more source

Mitochondrial diseases

open access: yesBiochimica et Biophysica Acta (BBA) - Bioenergetics, 2004
By convention, the term "mitochondrial diseases" refers to disorders of the mitochondrial respiratory chain, which is the only metabolic pathway in the cell that is under the dual control of the mitochondrial genome (mtDNA) and the nuclear genome (nDNA).
openaire   +2 more sources

Prevalence of nuclear and mitochondrial DNA mutations related to adult mitochondrial disease

open access: yesAnnals of Neurology, 2015
The prevalence of mitochondrial disease has proven difficult to establish, predominantly as a result of clinical and genetic heterogeneity. The phenotypic spectrum of mitochondrial disease has expanded significantly since the original reports that ...
G. Gorman   +12 more
semanticscholar   +1 more source

Mitochondrial Diseases

open access: yesJournal of Epilepsy Research, 1970
Mitochondria contain the respiratory chain enzyme complexes that carry out oxidative phosphorylation and produce the main part of cellular energy in the form of ATP. Although several proteins related with signalling, assembling, transporting, and enzymatic function can be impaired in mitochondrial diseases, most frequently the activity of the ...
openaire   +2 more sources

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