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Condition: Mitochondrial Disease

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Total 851 results found since Jan 2013.

Application and prospects of butylphthalide for the treatment of neurologic diseases.
CONCLUSIONS: The varied pharmacologic mechanisms of NBP involve many complex molecular mechanisms; however, there many unknown pharmacologic effects await further study. PMID: 31205106 [PubMed - in process]
Source: Chinese Medical Journal - June 18, 2019 Category: General Medicine Authors: Chen XQ, Qiu K, Liu H, He Q, Bai JH, Lu W Tags: Chin Med J (Engl) Source Type: research

Molecules, Vol. 24, Pages 2323: Advances in Biosynthesis, Pharmacology, and Pharmacokinetics of Pinocembrin, a Promising Natural Small-Molecule Drug
ng Yang Pinocembrin is one of the most abundant flavonoids in propolis, and it may also be widely found in a variety of plants. In addition to natural extraction, pinocembrin can be obtained by biosynthesis. Biosynthesis efficiency can be improved by a metabolic engineering strategy and a two-phase pH fermentation strategy. Pinocembrin poses an interest for its remarkable pharmacological activities, such as neuroprotection, anti-oxidation, and anti-inflammation. Studies have shown that pinocembrin works excellently in treating ischemic stroke. Pinocembrin can reduce nerve damage in the ischemic area and reduce mitochon...
Source: Molecules - June 23, 2019 Category: Chemistry Authors: Xiaoling Shen Yeju Liu Xiaoya Luo Zhihong Yang Tags: Review Source Type: research

Fulminant cerebral venous thrombosis associated with the m.3243A > G MELAS mutation: A new guise for an old disease
We describe a novel presentation of MELAS-syndrome with severe cerebral venous thrombosis (CVT) and inflammation with a vasculopathy that affects the venous system as well.
Source: Brain and Development - July 21, 2019 Category: Neurology Authors: Marc Nikolaus, Anna Tietze, Leonille Schweizer, Angela M. Kaindl, Werner Stenzel, Markus Schuelke, Ellen Knierim Tags: Case Report Source Type: research

Expanding and validating the biomarkers for mitochondrial diseases
In this study, we investigated a panel of blood biomarkers in a cohort of 123 mitochondrial patients, with prominent neurological and muscular manifestations. These biomarkers included creatine, fibroblast growth factor 21 (FGF21) and growth/differentiation factor 15 (GDF-15), and the novel cell free circulating-mtDNA (ccf-mtDNA). All biomarkers were significantly increased in the patient group. After stratification by the specific phenotypes, ccf-mtDNA  was significantly increased in the Mitochondrial Encephalomyopathy Lactic Acidosis Stroke-like episodes syndrome (MELAS) group, and FGF21 and GDF-15 were significantly el...
Source: Journal of Molecular Medicine - August 25, 2020 Category: Molecular Biology Source Type: research

Mitochondrial DNA m.3243A > G mutation rarely causes CADASIL-like phenotype.
Mitochondrial DNA m.3243A>G mutation rarely causes CADASIL-like phenotype. Neurobiol Aging. 2020 Aug 27;: Authors: Liao NY, Liao KK, Liao YC, Lee YC Abstract Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) are 2 monogenic cerebral small vessel diseases sharing several common clinical features including young stroke, migraine, and cognitive dysfunction. The aim of this study was to understand the role of MELAS in patients with CADASIL-like manifestations. We scr...
Source: Neurobiology of Aging - August 26, 2020 Category: Geriatrics Authors: Liao NY, Liao KK, Liao YC, Lee YC Tags: Neurobiol Aging Source Type: research

Ablation of TMEM126B protects against oxygen-glucose deprivation/reoxygenation-induced injuries of PC12  cells via maintaining mitochondrial anti-apoptotic functions.
Ablation of TMEM126B protects against oxygen-glucose deprivation/reoxygenation-induced injuries of PC12 cells via maintaining mitochondrial anti-apoptotic functions. Arch Biochem Biophys. 2020 Oct 16;:108634 Authors: Wei L, Fan Y, Wei L, He X, Yang J, Zheng X Abstract Ischemia reperfusion (I/R) injury is a key contributing factor to the pathogenic mechanism involved in cerebral infarction. Transmembrane protein 126b (TMEM126B), a mitochondrial complex I assembly factor, has been reported to have an intimate association with disease progression, but is little known in ischemia stroke. The present stu...
Source: Archives of Biochemistry and Biophysics - October 16, 2020 Category: Biochemistry Authors: Wei L, Fan Y, Wei L, He X, Yang J, Zheng X Tags: Arch Biochem Biophys Source Type: research

Uncoupling Protein 2 as a pathogenic determinant and therapeutic target in cardiovascular and metabolic diseases
Curr Neuropharmacol. 2021 Apr 20. doi: 10.2174/1570159X19666210421094204. Online ahead of print.ABSTRACTUncoupling protein 2 (UCP2) is a mitochondrial protein that acts as an anion carrier. It is involved in the regulation of several processes including mitochondrial membrane potential, generation of reactive oxygen species within the inner mitochondrial membrane and calcium homeostasis. UCP2 expression can be regulated at different levels: genetic (gene variants), transcriptional [by peroxisome proliferator-activated receptors (PPARs) and microRNAs], and post-translational. Experimental evidence indicates that activation ...
Source: Current Neuropharmacology - April 22, 2021 Category: Drugs & Pharmacology Authors: Rosita Stanzione Maurizio Forte Maria Cotugno Franca Bianchi Simona Marchitti Carla Letizia Busceti Francesco Fornai Speranza Rubattu Source Type: research

MELAS/LS Overlap Syndrome Associated With Mitochondrial DNA Mutations: Clinical, Genetic, and Radiological Studies
Discussion: Our study demonstrated that MTND genes are important mutation hot spots in MELAS/LS overlap syndrome. The follow-up is very important for the final diagnosis of overlap syndrome.
Source: Frontiers in Neurology - May 7, 2021 Category: Neurology Source Type: research

Linear cortical cystic lesions: Characteristic MR findings in MELAS patients
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a progressive neurodegenerative disorder with stroke-like lesions. The common MRI findings are gyral swelling and high signal intensity on T2WI/FLAIR images crossing the vascular territories. We have observed a linear cystic lesion and a laminar necrosis in the affected cortices of MELAS patients. Herein, we evaluated these cortical MRI findings in each subtype of mitochondrial disease.
Source: Brain and Development - May 25, 2021 Category: Neurology Authors: Hidetoshi Ishigaki, Noriko Sato, Yukio Kimura, Eri Takeshita, Hirofumi Komaki, Emiko Chiba, Yoko Shigemoto, Yu-ichi Goto, Madoka Mori-Yoshimura, Masayuki Sasaki Tags: Original article Source Type: research

The Protective Effect of Liquiritin in Hypoxia/Reoxygenation-Induced Disruption on Blood Brain Barrier
Conclusions: Studies have shown that LQ can promote cell proliferation, migration as well as angiogenesis, and reduce cell apoptosis, which may be related to its inhibition of oxidative and ER stress, and then maintain the integrity of BBB. Given that five differential proteins were found by protein analysis, future studies will revolve around the five differential proteins.
Source: Frontiers in Pharmacology - July 6, 2021 Category: Drugs & Pharmacology Source Type: research

Intratubular, Intracellular, and Mitochondrial Angiotensin II/AT1 (AT1a) Receptor/NHE3 Signaling Plays a Critical Role in Angiotensin II-Induced Hypertension and Kidney Injury
The objectives of this invited article are to review and discuss our recent findings that (a) circulating and intratubular Ang II is taken up by the proximal tubules via the (AT1) AT1a receptor-dependent mechanism, (b) intracellular administration of Ang II in proximal tubule cells or adenovirus-mediated overexpression of an intracellular Ang II fusion protein selectively in the mitochonria of the proximal tubules induces blood pressure responses, and (c) genetic deletion of AT1 (AT1a) receptors or the Na+/H+ exchanger 3 selectively in the proximal tubules decreases basal blood pressure and attenuates Ang II-induced hypert...
Source: Frontiers in Physiology - August 2, 2021 Category: Physiology Source Type: research

Mitochondrial diseases
MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is a mitochondrial encephalomyopathy characterized by stroke. It has been reported that 22% of patients with MELAS died before the age of 18 and 20.8% died on average 7.3 years after diagnosis. Here we report the autopsy findings of two adult-onset and one childhood-onset MELAS cases. We compared the autopsy findings of three MELAS (m.3243A>G) cases performed at this facility. (Case 1) A 38-year-old woman. She was diagnosed with MELAS at the age of 10.
Source: Neuromuscular Disorders - September 19, 2021 Category: Neurology Authors: A. Ishii, S. Ouchi, R. Matsuoka, A. Tamaoka, M. Noguchi Source Type: research