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Condition: Mitochondrial Disease

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Total 851 results found since Jan 2013.

The Most Promising Cancer-Fighter In Years
For a while now, I’ve been recommending a powerful antioxidant with the unpronounceable name, pyrroloquinoline quinine – or PQQ for short. And although I recommend this compound to almost everyone who comes to see me at my wellness clinic in South Florida because of its energy-giving qualities, I’ve also observed its extraordinary power to fight cancer. For years, I’ve seen PQQ work wonders with my older patients, because it keeps you feeling young by giving your cells extra energy. PQQ was first discovered back in 1979 by a team of Japanese scientists, who knew little about the substance, except that i...
Source: Al Sears, MD Natural Remedies - March 24, 2015 Category: Complementary Medicine Authors: Andrew Britton Tags: Anti-Aging antioxidants Cancer energy Source Type: news

Super Nutrient’s Liver Surprise
I was one of the first doctors to talk about the incredible benefits of the super-nutrient pyrroloquinoline quinone, or PQQ for short. I was also one of the first doctors to recommend it to patients. Now I recommend this essential nutrient and powerful antioxidant to almost everyone who comes to see me at my wellness clinic. Researchers have only just recently begun to understand the many important roles of PQQ on the body’s cellular processes. Not only does it possess extraordinary energy-giving qualities and have the power to ease nerve pain and battle Alzheimer’s, it has the potential to become the world’s stronge...
Source: Al Sears, MD Natural Remedies - June 10, 2015 Category: Complementary Medicine Authors: Dr. Al Sears Tags: Anti-Aging Cancer CoQ10 Source Type: news

BDNF Reduces Toxic Extrasynaptic NMDA Receptor Signaling via Synaptic NMDA Receptors and Nuclear-Calcium-Induced Transcription of inhba/Activin A
Publication date: Available online 13 August 2015 Source:Cell Reports Author(s): David Lau, C. Peter Bengtson, Bettina Buchthal, Hilmar Bading The health of neurons is critically dependent on the relative signaling intensities of survival-promoting synaptic and death-inducing extrasynaptic NMDA receptors. Here, we show that BDNF is a regulator of this balance and promotes neuroprotection by reducing toxic NMDA receptor signaling. BDNF acts by initiating synaptic NMDA-receptor/nuclear-calcium-driven adaptogenomics, leading to increased expression of inhibin β-A (inhba). Inhibin β-A (its homodimer is known as activi...
Source: Cell Reports - August 14, 2015 Category: Cytology Source Type: research

Hemiplegia and taking anabolic
We report the case of a young sportsman practicing bodybuilding as a recreation, 35 years old, without particular pathological history and having absorbed anabolic steroids for doping reasons: oxymetholone and methandrostenolone 1 tablet/D for 40 days before his stroke. Observation The clinical symptoms began to the awakening with the onset of functional impairment of the left side having recovered in 30minutes followed by a left total hemiplegia with facial central ipsilateral paralysis and swallowing disorders. CT at the same evening objectified a hypodensity in the territory of the right sylvian artery, which became he...
Source: Annals of Physical and Rehabilitation Medicine - October 2, 2015 Category: Rehabilitation Source Type: research

The many faces of melas
We report the case of a 76-year-old man (the second oldest diagnosed case to the authors' knowledge) whose case exemplifies the complexity of diagnosing and managing these patients including the complications of the disease.
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 14, 2015 Category: Neurosurgery Authors: AlMasri, O., Massey, T., Mukhopadhyay, D., Walters, R. J. Tags: Genetics, Epilepsy and seizures, Headache (including migraine), Infection (neurology), Muscle disease, Neuromuscular disease, Musculoskeletal syndromes Thur 21, Parallel session 5: Therapeutics Source Type: research

Autism in the Son of a Woman with Mitochondrial Myopathy and Dysautonomia: A Case Report
Conclusion Given emerging evidence that mitochondrial dysfunction, particularly in the electron transport chain needed for cellular energy production, is an underlying pathophysiological mechanism for some varieties of ASD, clinicians should have a high index of suspicion for mitochondrial disease, especially when they encounter a patient with unusual neurological or constitutional symptoms. The prevalence of mitochondrial disease in ASD patients may be as high as five percent, which means that it is not the “zebra”[27] diagnosis that it might be in a non-ASD patient, where prevalence is about 0.01 percent.10 Reference...
Source: Innovations in Clinical Neuroscience - October 9, 2015 Category: Neuroscience Authors: ICN Online Editor Tags: Anxiety Disorders Asperger's syndrome Autism Behavioral and Cognitive Neurology Case Report Current Issue Intellectual Disability Neurologic Systems and Symptoms Pervasive Developmental Disorders ASD autism spectrum disorder dysauton Source Type: research

SIRT3 Deacetylates Ceramide Synthases Molecular Bases of Disease
Experimental evidence supports the role of mitochondrial ceramide accumulation as a cause of mitochondrial dysfunction and brain injury after stroke. Herein, we report that SIRT3 regulates mitochondrial ceramide biosynthesis via deacetylation of ceramide synthase (CerS) 1, 2, and 6. Reciprocal immunoprecipitation experiments revealed that CerS1, CerS2, and CerS6, but not CerS4, are associated with SIRT3 in cerebral mitochondria. Furthermore, CerS1, -2, and -6 are hyperacetylated in the mitochondria of SIRT3-null mice, and SIRT3 directly deacetylates the ceramide synthases in a NAD+-dependent manner that increases enzyme ac...
Source: Journal of Biological Chemistry - January 22, 2016 Category: Chemistry Authors: Novgorodov, S. A., Riley, C. L., Keffler, J. A., Yu, J., Kindy, M. S., Macklin, W. B., Lombard, D. B., Gudz, T. I. Tags: Lipids Source Type: research

The phenotypic spectrum of fifty Czech m.3243A>G carriers
Conclusions Sufficient knowledge of the timeline of the natural course of MELAS syndrome may improve the prediction and management of symptoms in patients with this mitochondrial disease.
Source: Molecular Genetics and Metabolism - June 6, 2016 Category: Genetics & Stem Cells Source Type: research

FGF21 represses cerebrovascular aging via improving mitochondrial biogenesis and inhibiting p53 signaling pathway in an AMPK-dependent manner.
Abstract Cerebrovascular aging has a high relationship with stroke and neurodegenerative disease. In the present study, we evaluated the influence of fibroblast growth factor 21 (FGF21) on angiotensin (Ang II)-mediated cerebrovascular aging in human brain vascular smooth muscle cells (hBVSMCs). Ang II induced remarkable aging-phenotypes in hBVSMCs, including enhanced SA-β-gal staining and NBS1 protein expression. First, we used immunoblotting assay to confirm protein expression of FGF21 receptor (FGFR1) and the co-receptor β-Klotho in cultured hBVSMCs. Second, we found that FGF21 treatment partly prevented the a...
Source: Experimental Cell Research - June 26, 2016 Category: Cytology Authors: Wang XM, Xiao H, Liu LL, Cheng D, Li XJ, Si LY Tags: Exp Cell Res Source Type: research

The phenotypic spectrum of fifty Czech m.3243A & gt;G carriers
Conclusions Sufficient knowledge of the timeline of the natural course of MELAS syndrome may improve the prediction and management of symptoms in patients with this mitochondrial disease.
Source: Molecular Genetics and Metabolism - July 20, 2016 Category: Genetics & Stem Cells Source Type: research

The Role of Mitochondrial Aldehyde Dehydrogenase 2 (ALDH2) in Neuropathology and Neurodegeneration.
Authors: Chen CH, Joshi AU, Mochly-Rosen D Abstract Aldehydes-induced toxicity has been implicated in many neurodegenerative diseases. Exposure to reactive aldehydes from (1) alcohol and food metabolism; (2) environmental pollutants, including car, factory exhausts, smog, pesticides, herbicides; (3) metabolism of neurotransmitters, amino acids and (4) lipid peroxidation of biological membrane from excessive ROS, all contribute to 'aldehydic load' that has been linked to the pathology of neurodegenerative diseases. In particular, the α, β-unsaturated aldehydes derived from lipid peroxidation, 4-hydroxynonenal (4-H...
Source: Acta Neurologica Taiwanica - April 8, 2017 Category: Neurology Tags: Acta Neurol Taiwan Source Type: research

An Unusual Cause of Hyperglycemia
​BY CHRISTINE SHEELER; SEEMA ELAHI, MDA 46-year-old man presented to the ED with multiple complaints, including fatigue and dizziness for one day. His primary care physician had diagnosed him with diabetes the previous day, and had prescribed him an oral hypoglycemic as well as insulin. Neither, however, was started at the time of presentation. The patient had a medical history significant for HIV, with his most recent CD4 count above 500, and bilateral sensorineural hearing loss, which had progressively worsened since its initial onset in his 20s. His physical exam was unremarkable except for decreased hearing bilateral...
Source: The Case Files - April 13, 2017 Category: Emergency Medicine Tags: Blog Posts Source Type: research

Folic acid reduces doxorubicin ‐induced cardiomyopathy by modulating endothelial nitric oxide synthase
Abstract The use of doxorubicin (DOXO) as a chemotherapeutic drug has been hampered by cardiotoxicity leading to cardiomyopathy and heart failure. Folic acid (FA) is a modulator of endothelial nitric oxide (NO) synthase (eNOS), which in turn is an important player in diseases associated with NO insufficiency or NOS dysregulation, such as pressure overload and myocardial infarction. However, the role of FA in DOXO‐induced cardiomyopathy is poorly understood. The aim of this study was to test the hypothesis that FA prevents DOXO‐induced cardiomyopathy by modulating eNOS and mitochondrial structure and function. Male C57B...
Source: Journal of Cellular and Molecular Medicine - June 13, 2017 Category: Molecular Biology Authors: Yanti Octavia, Georgios Kararigas, Martine Boer, Ihsan Chrifi, Rinrada Kietadisorn, Melissa Swinnen, Hans Duimel, Fons K. Verheyen, Maarten M. Brandt, Daniela Fliegner, Caroline Cheng, Stefan Janssens, Dirk J. Duncker, An L. Moens Tags: Original Article Source Type: research

Folic acid reduces doxorubicin-induced cardiomyopathy by modulating endothelial nitric oxide synthase.
Abstract The use of doxorubicin (DOXO) as a chemotherapeutic drug has been hampered by cardiotoxicity leading to cardiomyopathy and heart failure. Folic acid (FA) is a modulator of endothelial nitric oxide (NO) synthase (eNOS), which in turn is an important player in diseases associated with NO insufficiency or NOS dysregulation, such as pressure overload and myocardial infarction. However, the role of FA in DOXO-induced cardiomyopathy is poorly understood. The aim of this study was to test the hypothesis that FA prevents DOXO-induced cardiomyopathy by modulating eNOS and mitochondrial structure and function. Male...
Source: J Cell Mol Med - June 13, 2017 Category: Molecular Biology Authors: Octavia Y, Kararigas G, de Boer M, Chrifi I, Kietadisorn R, Swinnen M, Duimel H, Verheyen FK, Brandt MM, Fliegner D, Cheng C, Janssens S, Duncker DJ, Moens AL Tags: J Cell Mol Med Source Type: research

Oral Consumption of Vitamin K2 for 8 Weeks Associated With Increased Maximal Cardiac Output During Exercise.
Conclusions • Although vitamin K2 supplementation has previously been reported to improve cardiovascular function in diseased patients, to the research team's knowledge, the current study is the first to report its potential in active individuals. More research is needed to fully evaluate the potential effects of the observed effects. PMID: 28646812 [PubMed - in process]
Source: Alternative Therapies in Health and Medicine - June 25, 2017 Category: Complementary Medicine Authors: McFarlin BK, Henning AL, Venable AS Tags: Altern Ther Health Med Source Type: research