Shannon Chiang, Zaklina Kovacevic, Sumit Sahni, Darius J.R. Lane, Angelica M. Merlot, Danuta S. Kalinowski, Michael L.-H. Huang, Des R. Richardson, Clinical Science Apr 22, 2016, 130 (11) 853-870; DOI: 10.1042/CS20160072
Monday, May 2, 2016
Sunday, May 1, 2016
University of Alabama utilizes Mawi’s iSWAB-Protein buccal cell collection device for the detection of the mitochondrial protein Frataxin
Mawi DNA Technologies / News. April 27, 2016
Dr. Jill Butler from Dr. Marek Napierala’s lab at the University of Alabama Stem Cell Institute used our iSWAB-Protein non-invasive sample collection system for biomarker detection in buccal cells.
Dr. Jill Butler from Dr. Marek Napierala’s lab at the University of Alabama Stem Cell Institute used our iSWAB-Protein non-invasive sample collection system for biomarker detection in buccal cells.
Friday, April 29, 2016
Role of neuroimaging in the diagnosis of hereditary cerebellar ataxias in childhood
Giulia Perucca, Nicolas Leboucq, Agathe Roubertie, François Rivier, Nicolas Menjot, Consuelo Valentini, Alain Bonafe, Journal of Neuroradiology, Available online 25 April 2016, ISSN 0150-9861, doi:10.1016/j.neurad.2016.03.006.
Friedreich ataxia (FA) is characterized on MRI bynormal morphology of the cerebellum and brainstem, withatrophy of the spinal cord . A rare involvementof dentate nuclei has been described. Cerebellaratrophy occurs late in the course of the disease.
Friedreich ataxia (FA) is characterized on MRI bynormal morphology of the cerebellum and brainstem, withatrophy of the spinal cord . A rare involvementof dentate nuclei has been described. Cerebellaratrophy occurs late in the course of the disease.
Thursday, April 28, 2016
Mitochondrial DNA damage induced autophagy, cell death, and disease
Bennett Van Houten, Senyene E. Hunter, and Joel N. Meyer; Frontiers in Bioscience, Landmark, 21, 42-54, January 1, 2016.
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Several human pathologies have been associated with free radical damage and subsequent mtDNA damage and mitochondrial dysfunction. These diseases include diabetes mellitus, liver diseases (e.g. hemochromatosis), cardiovascular disease (e.g. atherosclerosis) and several neurodegenerative diseases (e.g. Alzheimer’s, Parkinson’s Disease, Friedreich’s Ataxia and Huntington’s disease). In fact, multiple studies have linked human diseases associated with oxidative stress and loss of mitochondrial function to mtDNA damage
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Several human pathologies have been associated with free radical damage and subsequent mtDNA damage and mitochondrial dysfunction. These diseases include diabetes mellitus, liver diseases (e.g. hemochromatosis), cardiovascular disease (e.g. atherosclerosis) and several neurodegenerative diseases (e.g. Alzheimer’s, Parkinson’s Disease, Friedreich’s Ataxia and Huntington’s disease). In fact, multiple studies have linked human diseases associated with oxidative stress and loss of mitochondrial function to mtDNA damage
Wednesday, April 27, 2016
Genome Engineering with TALE and CRISPR Systems in Neuroscience
Han B. Lee, Brynn N. Sundberg, Ashley N. Sigafoos, and Karl J. Clark. Front Genet. 2016; 7: 47. doi: 10.3389/fgene.2016.00047
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These technologies mirror and extend beyond classic gene targeting methods contributing to the development of novel tools for basic and clinical neuroscience. In this Review, we discuss the recent development in genome engineering and potential applications of this technology in the field of neuroscience.
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These technologies mirror and extend beyond classic gene targeting methods contributing to the development of novel tools for basic and clinical neuroscience. In this Review, we discuss the recent development in genome engineering and potential applications of this technology in the field of neuroscience.
Tuesday, April 26, 2016
Neurodegenerative diseases and therapeutic strategies using iron chelators
Roberta J. Ward, David T. Dexter, Robert R. Crichton, Journal of Trace Elements in Medicine and Biology, Volume 31, July 2015, Pages 267-273, ISSN 0946-672X, doi: 10.1016/j.jtemb.2014.12.012.
Evidence for metal involvement in Parkinson's and Alzheimer's disease as well as Friedreich's ataxia and multiple sclerosis is presented. Preliminary results from trials of iron chelation therapy in these neurodegenerative diseases are reviewed. Considering the importance of metal ions in the normal functions of the human brain, it is not surprising that dysregulation of metal homeostasis should have harmful effects on brain function. The therapeutic utilisation of iron chelators in neurodegenerative diseases is still in its infancy.
Evidence for metal involvement in Parkinson's and Alzheimer's disease as well as Friedreich's ataxia and multiple sclerosis is presented. Preliminary results from trials of iron chelation therapy in these neurodegenerative diseases are reviewed. Considering the importance of metal ions in the normal functions of the human brain, it is not surprising that dysregulation of metal homeostasis should have harmful effects on brain function. The therapeutic utilisation of iron chelators in neurodegenerative diseases is still in its infancy.
Monday, April 25, 2016
Dual Role of ROS as Signal and Stress Agents: Iron Tips the Balance in favor of Toxic Effects
Elena Gammella, Stefania Recalcati, and Gaetano Cairo. Oxidative Medicine and Cellular Longevity, Volume 2016 (2016), Article ID 8629024, 9 pages, doi:10.1155/2016/8629024
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Abundant evidence shows that a number of neurodegenerative disorders are characterized by regional iron accumulation in particular areas of the central and/or peripheral nervous systems.
This is often caused by cellular iron redistribution and may result in iron-catalyzed Fenton chemistry.Friedrich's ataxia (FRDA) is a paradigmatic example because the disruption of iron homeostasis in this disease has been well defined. The disease results from loss of function mutations (most often triplet expansion) in the FXN gene that lead to decreased expression of frataxin, a mitochondrial iron-binding protein that interacts with proteins involved in the mitochondrial Fe-S cluster biogenesis. In patients, frataxin deficiency results in disruption of Fe-S cluster biosynthesis, severe mitochondrial iron overload, a hallmark of Fe-S defects, and increased sensitivity to oxidative stress.
OPEN ACCESS (Creative Commons Attribution License)
Abundant evidence shows that a number of neurodegenerative disorders are characterized by regional iron accumulation in particular areas of the central and/or peripheral nervous systems.
This is often caused by cellular iron redistribution and may result in iron-catalyzed Fenton chemistry.Friedrich's ataxia (FRDA) is a paradigmatic example because the disruption of iron homeostasis in this disease has been well defined. The disease results from loss of function mutations (most often triplet expansion) in the FXN gene that lead to decreased expression of frataxin, a mitochondrial iron-binding protein that interacts with proteins involved in the mitochondrial Fe-S cluster biogenesis. In patients, frataxin deficiency results in disruption of Fe-S cluster biosynthesis, severe mitochondrial iron overload, a hallmark of Fe-S defects, and increased sensitivity to oxidative stress.
Sunday, April 24, 2016
Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia
Dörte Poburski, Josefine Barbara Boerner, Michel Koenig, Michael Ristow, René Thierbach. Biology Open 2016 : doi: 10.1242/bio.017004
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Here, we have developed a new mammalian cell model employing the Cre/loxP recombination system to induce a homozygous or heterozygous frataxin knockout in mouse embryonic fibroblasts. Induction of Cre-mediated disruption by tamoxifen was successfully tested on RNA and protein levels. The robustness of the newly established system may additionally be used for a time-resolved study of pharmacological candidates in a HTS manner.
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Here, we have developed a new mammalian cell model employing the Cre/loxP recombination system to induce a homozygous or heterozygous frataxin knockout in mouse embryonic fibroblasts. Induction of Cre-mediated disruption by tamoxifen was successfully tested on RNA and protein levels. The robustness of the newly established system may additionally be used for a time-resolved study of pharmacological candidates in a HTS manner.
Saturday, April 23, 2016
Nrf2 activation in the treatment of neurodegenerative diseases: a focus on its role in mitochondrial bioenergetics and function
Noemí Esteras, Albena T. Dinkova-Kostova, Andrey Y. Abramov. Biological Chemistry. Volume 397, Issue 5, Pages 383–400, ISSN (Online) 1437-4315, ISSN (Print) 1431-6730, DOI: 10.1515/hsz-2015-0295, January 2016
As part of its cytoprotective activity, increasing evidence supports its role in metabolism and mitochondrial bioenergetics and function. Neurodegenerative diseases are excellent candidates for Nrf2-targeted treatments. Most neurodegenerative conditions such as Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, frontotemporal dementia and Friedreich’s ataxia are characterized by oxidative stress, misfolded protein aggregates, and chronic inflammation, the common targets of Nrf2 therapeutic strategies.
As part of its cytoprotective activity, increasing evidence supports its role in metabolism and mitochondrial bioenergetics and function. Neurodegenerative diseases are excellent candidates for Nrf2-targeted treatments. Most neurodegenerative conditions such as Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, frontotemporal dementia and Friedreich’s ataxia are characterized by oxidative stress, misfolded protein aggregates, and chronic inflammation, the common targets of Nrf2 therapeutic strategies.
Friday, April 22, 2016
Cardiac Serum Biomarkers in Friedreich Ataxia May Reflect Fibrosis, Myocyte Injury, and Degree of Hypertrophy
C. Bui, R.B. Wilson, D.R. Lynch, J.W. Rossano, O. Elci, K.Y. Lin, The Journal of Heart and Lung Transplantation, Volume 35, Issue 4, Supplement, April 2016, Page S172, ISSN 1053-2498, doi:10.1016/j.healun.2016.01.479.
We hypothesized that serum markers of cardiac injury, stress, fibrosis, and inflammation are higher in FA subjects than non-FA controls, and these markers correlate with echocardiographic markers of cardiomyopathy.
We hypothesized that serum markers of cardiac injury, stress, fibrosis, and inflammation are higher in FA subjects than non-FA controls, and these markers correlate with echocardiographic markers of cardiomyopathy.
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