Results 21 to 30 of about 533,276 (294)
Mitochondrial disease in children [PDF]
Journal of Internal Medicine, 2020 AbstractMitochondrial disease presenting in childhood is characterized by clinical, biochemical and genetic complexity. Some children are affected by canonical syndromes, but the majority have nonclassical multisystemic disease presentations involving virtually any organ in the body.
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Journal of Pharmacological Sciences, 2010 We investigated the effects of YC-1, an activator of soluble guanylyl cyclase (sGC), on voltage-dependent K+ (Kv) channels in smooth muscle cells from freshly isolated rabbit coronary arteries by using the whole-cell patch clamp technique. YC-1 inhibited
Won Sun Park +9 more
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Mitochondrial Dynamics in Mitochondrial Diseases
Diseases, 2016 Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial network quality control.
Juan Suárez-Rivero +9 more
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Drug 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
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Marine Drugs, 2018 Echinochrome A (EchA) is a marine bioproduct extracted from sea urchins having antioxidant, antimicrobial, anti-inflammatory, and chelating effects, and is the active component of the clinical drug histochrome.
Hyoung Kyu Kim +11 more
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Variable effects of omaveloxolone (RTA408) on primary fibroblasts with mitochondrial defects
Frontiers in Molecular Biosciences, 2022 Omaveloxolone (RTA408) is a second-generation oleanane triterpenoid Nrf2 inducer with antioxidant and anti-inflammatory properties and was reported to improve mitochondrial bioenergetics.
Madleen Zighan +10 more
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Journal 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 ...
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Mitochondrial disease in flies
Biochimica 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).
Jacobs, Howard T. +5 more
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Mitochondrial diseases and epilepsy [PDF]
Epilepsia, 2012 SummaryThe mitochondrial respiratory chain is the final common pathway for energy production. Defects affecting this pathway can give rise to disease that presents at any age and affects any tissue. However, irrespective of genetic defect, epilepsy is common and there is a significant risk of status epilepticus.
Laurence A, Bindoff, Bernt A, Engelsen
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Treatment for mitochondrial diseases [PDF]
Reviews in the Neurosciences, 2020 Abstract Mitochondrial diseases are predominantly caused by mutations of mitochondrial or nuclear DNA, resulting in multisystem defects. Current treatments are largely supportive, and the disorders progress relentlessly. Nutritional supplements, pharmacological agents and physical therapies have been used in different clinical trials ...
Tongling, Liufu, Zhaoxia, Wang
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