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Can vitamins and dietary supplements benefit patients with mitochondrial disease?

(Children's Hospital of Philadelphia) Defects in mitochondria, the tiny structures that power cells by functioning as biological batteries, cause an array of complex disorders that can affect any and all organs and systems. In the absence of validated, effective drug treatments, patients with mitochondrial disease often take a variety of vitamins and supplements, substances that are largely unstandardized, unregulated, and unproven. Experts in the field are calling for systematic studies to evaluate nutritional interventions.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

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Conclusion: CypD stimulates mPTP excessive opening, subsequently causing endoplasmic reticulum (ER) stress via p38 MAPK activation, and resultes in enhanced SREBP‐1c transcription and hepatic steatosis. This article is protected by copyright. All rights reserved.
Source: Hepatology - Category: Internal Medicine Authors: Tags: Steatohepatitis and Metabolic Liver Disease Source Type: research
Authors: Bryant KG, Chae YC, Martinez RL, Gordon JC, Elokely KM, Kossenkov AV, Grant S, Childers WE, Abou-Gharbia M, Altieri DC Abstract Reprogramming of mitochondrial functions sustains tumor growth and may provide therapeutic opportunities. Here, we targeted the protein folding environment in mitochondria by coupling a purine-based inhibitor of the molecular chaperone Heat Shock Protein-90 (Hsp90), PU-H71 to the mitochondrial-targeting moiety, triphenylphosphonium (TPP). Binding of PU-H71-TPP to ADP-Hsp90, Hsp90 co-chaperone complex or mitochondrial Hsp90 homolog, TRAP1 involved hydrogen bonds, π-π stacking...
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research
Publication date: March 2018 Source:Biomedicine & Pharmacotherapy, Volume 99 Author(s): Dharmendra Choudhary, Sulekha Adhikary, Naseer Ahmad, Priyanka Kothari, Ashwni Verma, Prabodh Kumar Trivedi, Prabhat Ranjan Mishra, Ritu Trivedi Withaferin A (WFA), a highly oxygenated withanolide is used for anti-osteoporotic, fracture healing, obesity control as medicine and dietary supplement in Ayurveda and Unani medicine but its potential remains to be investigate for the osteoarthritis studies. In the present study, chondro-protective effects of WFA, under in vitro and in vivo conditions were evaluated. In-vitro pharmacologic...
Source: Biomedicine and Pharmacotherapy - Category: Drugs & Pharmacology Source Type: research
Publication date: 15 February 2018 Source:Veterinary Parasitology, Volume 251 Author(s): Pilar Alda, Manon Lounnas, Antonio Alejandro Vázquez, Rolando Ayaqui, Manuel Calvopiña, Maritza Celi-Erazo, Robert T. Dillon, Philippe Jarne, Eric S. Loker, Flavia Caroll Muñiz Pareja, Jenny Muzzio-Aroca, Alberto Orlando Nárvaez, Oscar Noya, Luiggi Martini Robles, Richar Rodríguez-Hidalgo, Nelson Uribe, Patrice David, Jean-Pierre Pointier, Sylvie Hurtrez-Boussès A molecular tool described here allows in one step for specific discrimination among three cryptic freshwater snail species (genus Ga...
Source: Veterinary Parasitology - Category: Veterinary Research Source Type: research
PGC-1α ameliorates kidney fibrosis in mice with diabetic kidney disease through an antioxidative mechanism. Mol Med Rep. 2018 Jan 16;: Authors: Zhang L, Liu J, Zhou F, Wang W, Chen N Abstract The production of reactive oxygen species (ROS) is a common phenomenon in podocyte impairment, which leads to the irreversible progression of chronic kidney diseases, such as diabetic kidney disease (DKD). Previous research has indicated that peroxisome proliferator‑activated receptor γ (PPARγ) coactivator‑1α (PGC‑1α) participates in mitochondrial biogenesis and energy metabolis...
Source: Molecular Medicine Reports - Category: Molecular Biology Tags: Mol Med Rep Source Type: research
ABSTRACT Rotenone is a common pesticide and has been reported as one of the risk factors for Parkinson disease. Rotenone can cause neuronal death or apoptosis through inducing oxidative injury and inhibiting mitochondrial function. As a natural polyphenolic compound, resveratrol possesses the anti‐oxidant capacity and neuroprotective effect. However, the mechanism underlying the neuroprotective effect of resveratrol against rotenone‐induced neurotoxicity remains elusive. Here, we treated PC12 cells with rotenone to induce neurotoxicity, and the neurotoxic cells were subjected to resveratrol treatment. The CCK8 and LDH ...
Source: The Anatomical Record Part A: Discoveries in Molecular, Cellular, and Evolutionary Biology - Category: Molecular Biology Authors: Tags: Full Length Article Source Type: research
ConclusionsThus, to conclude, this report emphasizes the diagnostic value of FGF21 assay in this disorder. It extends the phenotype associated with the founder mutation inRMND1 gene in patients from South Asia.
Source: Indian Journal of Pediatrics - Category: Pediatrics Source Type: research
ConclusionsThese findings suggest that sex-specific decrease in neuroactive steroid levels in male diabetic animals may cause an alteration in their mitochondrial function that in turn might impact in axonal transport, contributing to the sex difference observed in diabetic neuropathy.
Source: Biology of Sex Differences - Category: Biology Source Type: research
Key Clinical Message Later‐onset congenital central hypoventilation syndrome (LO‐CCHS) does not present only breathing problems but can be present as episodic multiple organs involvement. Our unique case demonstrated LO‐CCHS should be considered in the differential diagnosis of mitochondrial diseases and having nontypical polysomnography result. Later‐onset congenital central hypoventilation syndrome (LO‐CCHS) does not present only breathing problems but can be present as episodic multiple organs involvement. Our unique case demonstrated LO‐CCHS should be considered in the differential diagnosis of mitochondri...
Source: Clinical Case Reports - Category: General Medicine Authors: Tags: Case Report Source Type: research
Contribution of Mössbauer spectroscopy to the investigation of Fe/S biogenesis. J Biol Inorg Chem. 2018 Jan 19;: Authors: Garcia-Serres R, Clémancey M, Latour JM, Blondin G Abstract Fe/S cluster biogenesis involves a complex machinery comprising several mitochondrial and cytosolic proteins. Fe/S cluster biosynthesis is closely intertwined with iron trafficking in the cell. Defects in Fe/S cluster elaboration result in severe diseases such as Friedreich ataxia. Deciphering this machinery is a challenge for the scientific community. Because iron is a key player, 57Fe-Mössbauer spectrosc...
Source: Journal of Biological Inorganic Chemistry : JBIC - Category: Chemistry Authors: Tags: J Biol Inorg Chem Source Type: research
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