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Source: Mitochondrion
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Total 25 results found since Jan 2013.
Metabolic abnormality in acute stroke-like lesion and its relationship with focal cerebral blood flow in patients with MELAS: Evidence from proton MR spectroscopy and arterial spin labeling
Publication date: Available online 27 June 2021Source: MitochondrionAuthor(s): Rong Wang, Bin Hu, Chong Sun, Daoying Geng, Jie Lin, Yuxin Li
Source: Mitochondrion - June 27, 2021 Category: Biochemistry Source Type: research
Optical coherence tomography as a possible tool to monitor and predict disease progression in mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
Publication date: Available online 19 November 2020Source: MitochondrionAuthor(s): Akihiro Shinkai, Yasuhiro Shinmei, Kiriko Hirooka, Yoshiaki Tagawa, Kayoko Nakamura, Shinki Chin, Susumu Ishida
Source: Mitochondrion - November 19, 2020 Category: Biochemistry Source Type: research
Altered spontaneous brain activity at attack and remission stages in patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS): beyond stroke-like lesions
Publication date: Available online 15 July 2020Source: MitochondrionAuthor(s): Rong Wang, Yuxin Li, Jie Lin, Chong Sun, Ne Chen, Weixingzi Xu, Bin Hu, Xueling Liu, Daoying Geng, Liqin Yang
Source: Mitochondrion - July 15, 2020 Category: Biochemistry Source Type: research
Mitochondrial Function and Brain Metabolic Score (BMS) in Ischemic Stroke: Evaluation of “Neuroprotectants” Safety and Efficacy
Publication date: Available online 29 November 2019Source: MitochondrionAuthor(s): Avraham Mayevsky, Hofit Kutai-Asis, Michael TolmasovAbstractThe initial and significant event developed in ischemic stroke is the sudden decrease in blood flow and oxygen supply to brain tissue, leading to dysfunction of the mitochondria. Many attempts were and are being made to develop new drugs and treatments that will save the ischemic brain, but the efficacy is not optimal and in many patients, irreversible damage to the brain will persist. We review a unique approach to evaluate mitochondrial function and microcirculatory hemodynamic in...
Source: Mitochondrion - November 30, 2019 Category: Biochemistry Source Type: research
Cerebrospinal fluid ATP as a potential biomarker in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes (MELAS)
Publication date: Available online 19 November 2019Source: MitochondrionAuthor(s): Takamasa Nukui, Atsushi Matsui, Hideki Niimi, Mamoru Yamamoto, Noriyuki Mastuda, Jin-Lan Piao, Kyo Noguchi, Isao Kitajima, Yuji NakastujiAbstractMitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is caused by defective oxidative phosphorylation in the cerebral parenchyma, cerebral blood vessels, and leptomeningeal tissue. Although increased serum and cerebrospinal fluid (CSF) lactate level has been used as a diagnostic biomarker in patients with MELAS, no biomarkers reflecting disease activity exist. Since we hav...
Source: Mitochondrion - November 20, 2019 Category: Biochemistry Source Type: research
Homozygous R627W mutations in POLG cause mitochondrial DNA depletion leading to encephalopathy, seizures and stroke-like episodes
Publication date: Available online 16 August 2019Source: MitochondrionAuthor(s): Arumugam Paramasivam, Challa Venkatapathi, Gampa Sandeep, Angamuthu K. Meena, Megha S. Uppin, Swati Mohapatra, Robert D.S. Pitceathly, Kumarasamy ThangarajAbstractMutations in the mitochondrial DNA maintenance gene POLG (DNA Polymerase Gamma, Catalytic Subunit), encoding mitochondrial DNA polymerase gamma (pol γ), are associated with an extremely broad phenotypic spectrum. We identified homozygous POLG c.1879C>T; p.R627W mutations in two siblings from a consanguineous South Asian family following targeted resequencing of 75 nuclear-encoded mi...
Source: Mitochondrion - August 17, 2019 Category: Biochemistry Source Type: research
Apomorphine rescues reactive oxygen species-induced apoptosis of fibroblasts with mitochondrial disease
In conclusion, apomorphine rescued fibroblasts from cell death under oxidative stress and improved the mitochondrial respiratory activity and appears to be potentially useful for treating mitochondrial disease.
Source: Mitochondrion - July 28, 2019 Category: Biochemistry Source Type: research
A urinary biosignature for mitochondrial myopathy, encephalopathy, lactic acidosis and stroke like episodes (MELAS)
Publication date: Available online 19 February 2018Source: MitochondrionAuthor(s): Karien Esterhuizen, J. Zander Lindeque, Shayne Mason, Francois H. van der Westhuizen, Anu Suomalainen, Anna H. Hakonen, Christopher J. Carroll, Richard J. Rodenburg, Paul B. de Laat, Mirian C.H. Janssen, Jan A.M. Smeitink, Roan LouwAbstractWe used a comprehensive metabolomics approach to study the altered urinary metabolome of two mitochondrial myopathy, encephalopathy lactic acidosis and stroke like episodes (MELAS) cohorts carrying the m.3243A>G mutation.The first cohort were used in an exploratory phase, identifying 36 metabolites that we...
Source: Mitochondrion - July 11, 2018 Category: Biochemistry Source Type: research
Involvement of mitochondrial haplogroups in myocardial infarction and stroke: A case-control study in Castile and Leon (Spain) population
Publication date: Available online 16 December 2017Source: MitochondrionAuthor(s): Miriam Umbria, Amanda Ramos, Jennifer Caner, Tomás Vega, José Eugenio Lozano, Cristina Santos, Maria Pilar AlujaAbstractThere are strong evidences that common mitochondrial DNA (mtDNA) haplogroups may influence the pathogenesis of cardiovascular diseases (CVDs). In this matched case-control study, we investigate the association between mtDNA haplogroups and two CVDs, myocardial infarction (MI) and stroke, and classical cardiovascular risk factors.Data obtained show that haplogroup H constitute a susceptibility risk factor for MI (p =&...
Source: Mitochondrion - July 11, 2018 Category: Biochemistry Source Type: research
A urinary biosignature for mitochondrial myopathy, encephalopathy, lactic acidosis and stroke like episodes (MELAS)
Publication date: Available online 19 February 2018Source: MitochondrionAuthor(s): Karien Esterhuizen, J. Zander Lindeque, Shayne Mason, Francois H. van der Westhuizen, Anu Suomalainen, Anna H. Hakonen, Christopher J. Carroll, Richard J. Rodenburg, Paul B. de Laat, Mirian C.H. Janssen, Jan A.M. Smeitink, Roan LouwAbstractWe used a comprehensive metabolomics approach to study the altered urinary metabolome of two mitochondrial myopathy, encephalopathy lactic acidosis and stroke like episodes (MELAS) cohorts carrying the m.3243A>G mutation.The first cohort were used in an exploratory phase, identifying 36 metabolites that we...
Source: Mitochondrion - July 5, 2018 Category: Biochemistry Source Type: research
Involvement of mitochondrial haplogroups in myocardial infarction and stroke: A case-control study in Castile and Leon (Spain) population
Publication date: Available online 16 December 2017Source: MitochondrionAuthor(s): Miriam Umbria, Amanda Ramos, Jennifer Caner, Tomás Vega, José Eugenio Lozano, Cristina Santos, Maria Pilar AlujaAbstractThere are strong evidences that common mitochondrial DNA (mtDNA) haplogroups may influence the pathogenesis of cardiovascular diseases (CVDs). In this matched case-control study, we investigate the association between mtDNA haplogroups and two CVDs, myocardial infarction (MI) and stroke, and classical cardiovascular risk factors.Data obtained show that haplogroup H constitute a susceptibility risk factor for MI (p =&...
Source: Mitochondrion - July 5, 2018 Category: Biochemistry Source Type: research
A urinary biosignature for mitochondrial myopathy, encephalopathy, lactic acidosis and stroke like episodes (MELAS)
Publication date: Available online 19 February 2018 Source:Mitochondrion Author(s): Karien Esterhuizen, J. Zander Lindeque, Shayne Mason, Francois H. van der Westhuizen, Anu Suomalainen, Anna H. Hakonen, Christopher J. Carroll, Richard J. Rodenburg, Paul B. de Laat, Mirian C.H. Janssen, Jan A.M. Smeitink, Roan Louw We used a comprehensive metabolomics approach to study the altered urinary metabolome of two mitochondrial myopathy, encephalopathy lactic acidosis and stroke like episodes (MELAS) cohorts carrying the m.3243A>G mutation. The first cohort were used in an exploratory phase, identifying 36 metabolites that...
Source: Mitochondrion - February 20, 2018 Category: Biochemistry Source Type: research
Involvement of mitochondrial haplogroups in myocardial infarction and stroke: A case-control study in Castile and Leon (Spain) population
Publication date: Available online 16 December 2017 Source:Mitochondrion Author(s): Miriam Umbria, Amanda Ramos, Jennifer Caner, Tomás Vega, José Eugenio Lozano, Cristina Santos, Maria Pilar Aluja There are strong evidences that common mitochondrial DNA (mtDNA) haplogroups may influence the pathogenesis of cardiovascular diseases (CVDs). In this matched case-control study, we investigate the association between mtDNA haplogroups and two CVDs, myocardial infarction (MI) and stroke, and classical cardiovascular risk factors. Data obtained show that haplogroup H constitute a susceptibility risk factor for MI (p=0.001; OR=2...
Source: Mitochondrion - December 17, 2017 Category: Biochemistry Source Type: research
Identification of FASTKD2 compound heterozygous mutations as the underlying cause of autosomal recessive MELAS-like syndrome
This study examined a Korean MELAS-like syndrome patient with seizure, stroke-like episode, and optic atrophy. Target sequencing of whole mtDNA and 73 nuclear genes identified compound heterozygous mutations p.R205X and p.L255P in the FASTKD2. Each of his unaffected parents has one of the two mutations, and both mutations were not found in 302 controls. FASTKD2 encodes a FAS-activated serine-threonine (FAST) kinase domain 2 which locates in the mitochondrial inner compartment. A FASTKD2 nonsense mutation was once reported as the cause of a recessive infantile mitochondrial encephalomyopathy. The present case showed relativ...
Source: Mitochondrion - May 10, 2017 Category: Biochemistry Source Type: research
No evidence of ischemia in stroke-like lesions of mitochondrial POLG encephalopathy
Publication date: Available online 10 November 2016 Source:Mitochondrion Author(s): Charalampos Tzoulis, Eilen Henriksen, Hrvoje Miletic, Laurence A. Bindoff Stroke-like lesions are characteristically associated with mitochondrial encephalopathies such as those caused by mutations of polymerase gamma (POLG) and the m.3243A>G mitochondrial DNA (mtDNA) mutation. The combination of acute clinical onset, MRI and pathological abnormalities, have led to the suggestion that these lesions are ischemic. Here, we sought to determine the role of ischemia in the pathogenesis of mitochondrial stroke-like lesions. We performed a...
Source: Mitochondrion - November 10, 2016 Category: Biochemistry Source Type: research
