Wednesday, April 24, 2013
Scientists Find Way to Turn Stem Cells Into Brain Cells
Scientists Find Way to Turn Stem Cells Into Brain Cells. Jason Koebler, U.S. News & World. April 23, 2013.
Saturday, April 20, 2013
New scholarship research into Friedreich Ataxia, heart and eye dysfunction
CERA student receives prestigious Gustav Nossal Scholarship for his research into Friedreich Ataxia .
CERA (Centre for Eye Research Australia).09 April, 2013
Duncan is a PhD student in CERA's Neuroregeneration Research Unit, he uses stem cells generated in the laboratory from FRDA patients' own skin, to grow certain types of cells found in the heart and eye. These cells will be used to better understand the pathology of FRDA, to conduct basic research on the disease and to test new drugs, prior to conducting clinical trials.
CERA (Centre for Eye Research Australia).09 April, 2013
Duncan is a PhD student in CERA's Neuroregeneration Research Unit, he uses stem cells generated in the laboratory from FRDA patients' own skin, to grow certain types of cells found in the heart and eye. These cells will be used to better understand the pathology of FRDA, to conduct basic research on the disease and to test new drugs, prior to conducting clinical trials.
Gene Therapy for Rare Diseases: Summary of a National Institutes of Health Workshop, September 13, 2012
Gene Therapy for Rare Diseases: Summary of a National Institutes of Health Workshop, September 13, 2012 . Marina O'Reilly, Donald B. Kohn, Jeffrey Bartlett, Janet Benson, Philip J. Brooks, Barry J. Byrne, Carlos Camozzi, Kenneth Cornetta, Ronald G. Crystal, Yuman Fong, Linda Gargiulo, Rashmi Gopal-Srivastava, Katherine A. High, Samuel G. Jacobson, Robert C. Jambou, Maureen Montgomery, Eugene Rosenthal, R. Jude Samulski, Sonia I. Skarlatos, Brian Sorrentino, James M. Wilson, Yun Xie, and Jacqueline Corrigan-Curay. Human Gene Therapy. April 2013, 24(4): 355-362. doi:10.1089/hum.2013.064.
Gene therapy has shown clinical efficacy for several rare diseases, using different approaches and vectors.
Gene therapy has shown clinical efficacy for several rare diseases, using different approaches and vectors.
Monday, April 15, 2013
Of flies and men: insights on organismal metabolism from fruit flies
Of flies and men: insights on organismal metabolism from fruit flies. Akhila Rajan and Norbert Perrimon; BMC Biology 2013, 11:38 doi:10.1186/1741-7007-11-38
OPEN ACCESS, FULL TEXT PDF
Altered lipid metabolism in human neurodegenerative disease models
Given the energy needs of neuronal cells, it is not surprising that deficits in energy metabolism manifest themselves most prominently in neuronal cell types. Genes that play a role in lipid homeostasis and mitochondrial function have been linked to adult onset neurodegeneration and have been extensively reviewed elsewhere [4,51,52]. Here we discuss insights obtained from a fly model of Friedreich's ataxia (FRDA) [53]. FRDA is the most common form of an autosomal recessive neurodegenerative disease affecting the central and peripheral nervous systems. It is caused by reduced expression of the mitochondrial protein frataxin, whose deficiency affects citric acid cycle function. Diabetes is a typical symptom of FRDA patients, and electron microscopic analysis of the neurons and cardiac muscles in mouse models shows an increase in lipid droplets [54], suggesting that there may be changes in lipid metabolism. To pursue further the role played by abnormal lipid metabolism in FRDA pathogenesis, Drosophila frataxin was removed from glial cells (neuronal support cells) by RNA interference (RNAi). This resulted in increased lipid droplet accumulation in glial cells and increased sensitivity to oxidative insults, neurodegeneration and impairment in locomotor activity. Interestingly, overexpression of Glial Lazarillo (GLaz) - the Drosophila homolog of human apolipoprotein D, a carrier protein of lipids - confers a protective effect on the Frataxin-RNAi flies. These studies suggest for the first time a specific requirement for frataxin in glial cells, and open the possibility that the control of lipid metabolism by apolipoproteins could represent a new strategy for the treatment of FRDA patients.
OPEN ACCESS, FULL TEXT PDF
Altered lipid metabolism in human neurodegenerative disease models
Given the energy needs of neuronal cells, it is not surprising that deficits in energy metabolism manifest themselves most prominently in neuronal cell types. Genes that play a role in lipid homeostasis and mitochondrial function have been linked to adult onset neurodegeneration and have been extensively reviewed elsewhere [4,51,52]. Here we discuss insights obtained from a fly model of Friedreich's ataxia (FRDA) [53]. FRDA is the most common form of an autosomal recessive neurodegenerative disease affecting the central and peripheral nervous systems. It is caused by reduced expression of the mitochondrial protein frataxin, whose deficiency affects citric acid cycle function. Diabetes is a typical symptom of FRDA patients, and electron microscopic analysis of the neurons and cardiac muscles in mouse models shows an increase in lipid droplets [54], suggesting that there may be changes in lipid metabolism. To pursue further the role played by abnormal lipid metabolism in FRDA pathogenesis, Drosophila frataxin was removed from glial cells (neuronal support cells) by RNA interference (RNAi). This resulted in increased lipid droplet accumulation in glial cells and increased sensitivity to oxidative insults, neurodegeneration and impairment in locomotor activity. Interestingly, overexpression of Glial Lazarillo (GLaz) - the Drosophila homolog of human apolipoprotein D, a carrier protein of lipids - confers a protective effect on the Frataxin-RNAi flies. These studies suggest for the first time a specific requirement for frataxin in glial cells, and open the possibility that the control of lipid metabolism by apolipoproteins could represent a new strategy for the treatment of FRDA patients.
Sunday, April 14, 2013
Lysine deacetylases and mitochondrial dynamics in neurodegeneration.
Lysine deacetylases and mitochondrial dynamics in neurodegeneration. Pedro Guedes-Dias, Jorge M.A. Oliveira; Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Available online 8 April 2013
Keywords: Mitochondria, HDAC, sirtuins, mitochondrial dynamics, biogenesis, mitophagy.
Keywords: Mitochondria, HDAC, sirtuins, mitochondrial dynamics, biogenesis, mitophagy.
Saturday, April 13, 2013
The L-Cysteine Desulfurase NFS1 Is Localized in the Cytosol where it Provides the Sulfur for Molybdenum Cofactor Biosynthesis in Humans
The L-Cysteine Desulfurase NFS1 Is Localized in the Cytosol where it Provides the Sulfur for Molybdenum Cofactor Biosynthesis in Humans,Zvonimir Marelja, Mita Mullick Chowdhury, Carsten Dosche, Carsten Hille, Otto Baumann, Hans-Gerd Löhmannsröben, Silke Leimkühler; PLoS ONE 8(4): e60869. doi:10.1371/journal.pone.0060869
Fifth Congress of the International BioIron Society (IBIS) (BioIron 2013) at University College London.
Fifth Congress of the International BioIron Society (IBIS) (BioIron 2013) at
University College London. 2013 Program Book - International BioIron Society, April 14-18
Podium #36
IRP-1 CONTROLS IRON METABOLISM IN FRATAXIN-DEFICIENT TISSUES
Alain Martelli, PhD, Stéphane Schmucker, PhD, Laurence Reutenauer, Hervé Puy, Prof., Bruno Galy, PhD, Matthias
Hentze, Prof. and Hélène Puccio, Research Director
Podium #47
ABNORMAL BODY IRON DISTRIBUTION AND ERYTHROPOIESIS IN A NOVEL MOUSE MODEL WITH INDUCIBLE GAIN
OF IRON REGULATORY PROTEIN (IRP) -1 FUNCTION
Daniela Casarrubea, MSc, Lydie Viatte, PhD, Tina Hallas, Aparna Vasanthakumar, PhD, Richard S. Eisenstein, PhD, Klaus
Schümann, PhD, Matthias W. Hentze, PhD and Bruno Galy, PhD
Podium #66
IDENTIFICATION OF NONFERRITIN MITOCHONDRIAL IRON DEPOSITS IN A MOUSE MODEL OF FRIEDREICH ATAXIA
Des R. Richardson, M. Whitnall, Y. Suryo Rahmant, M.L.H. Huang, F. Saletta, H.C. Lok, L. Gutierrez, F.J. Lazaro, A.J.
Fleming, T.G. St Pierre, M.R. Mikhael and P. Ponka
Poster #34
MITOCHONDRIAL PROTEIN, FRATAXIN, IS DOWNREGULATED IN HEMODIALYSIS PATIENTS
Takeshi Nakanishi, MD, PhD; Yukiko Hasuike, MD,PhD; Soshi Yorifuji, MD; Ayako Matsumoto, MD; Mana Yahiro, MD, PhD;
Aritoshi Kida, MD; Yasuyuki Nagasawa, MD, PhD; Takahiro Kuragano, MD, PhD
Poster #35
FRATAXIN IS A REGULATOR OF THE FE-S BIOGENESIS
Salvatore Adinolfi, biologist
Poster #39
MITOCHONDRIA-TARGETED IRON CHELATORS FOR THE MONITORING AND ADJUSTMENT OF CELLULAR LABILE
IRON POOLS
Vincenzo Abbate, PhD; Robert Hider, PhD; Charareh Pourzand, PhD; Olivier Reelfs, PhD
Poster #62
DOWN REGULATION OF RESPIRATORY ENZYMES IN FRATAXIN DEFICIENT YEAST IS MEDIATED BY THE
METABOLIC REGULATORS ADR1 AND CTH2
David Alsina, Armando Moreno-Cermeño, Elia Obis, Joaquim Ros and Jordi Tamarit
Poster #64
DEFINING THE ARCHITECTURE OF THE MITOCHODRIAL IRON-SULFUR CLUSTER ASSEMBLY MACHINERY
Belinda Galean
Poster #214
A CELL MODEL FOR FRIEDREICH ATAXIA USING DORSAL ROOT GANGLIA NEURONS
Stefka Mincheva-Tasheva, PhD, Elia Obis, Joaquim Ros, PhD and Jordi Tamarit, PhD
Poster #231
NOVEL LIGHT-ACTIVATED CAGED IRON CHELATORS: TARGETED PRODRUGS FOR IRON RELATED-DISORDERS
Benjamin Young, MPharm, Olivier Reelfs, PhD, DSc, Asma Aroun, PhD, Seyed Ali Miri, BSc, Magnus Hoffmann, BSc,
Charareh Pourzand, PhD, DSc and Ian Eggleston
Poster #273
A PRIMARY CULTURE OF CARDIOMYOCYTES WITH FRATAXIN DEFICIENCY EXHIBITS A METABOLIC SWITCH
BEFORE IRON DISARRANGEMENTS
Elia Obis, David Alsina, Joaquim Ros and Jordi Tamarit
University College London. 2013 Program Book - International BioIron Society, April 14-18
Podium #36
IRP-1 CONTROLS IRON METABOLISM IN FRATAXIN-DEFICIENT TISSUES
Alain Martelli, PhD, Stéphane Schmucker, PhD, Laurence Reutenauer, Hervé Puy, Prof., Bruno Galy, PhD, Matthias
Hentze, Prof. and Hélène Puccio, Research Director
Podium #47
ABNORMAL BODY IRON DISTRIBUTION AND ERYTHROPOIESIS IN A NOVEL MOUSE MODEL WITH INDUCIBLE GAIN
OF IRON REGULATORY PROTEIN (IRP) -1 FUNCTION
Daniela Casarrubea, MSc, Lydie Viatte, PhD, Tina Hallas, Aparna Vasanthakumar, PhD, Richard S. Eisenstein, PhD, Klaus
Schümann, PhD, Matthias W. Hentze, PhD and Bruno Galy, PhD
Podium #66
IDENTIFICATION OF NONFERRITIN MITOCHONDRIAL IRON DEPOSITS IN A MOUSE MODEL OF FRIEDREICH ATAXIA
Des R. Richardson, M. Whitnall, Y. Suryo Rahmant, M.L.H. Huang, F. Saletta, H.C. Lok, L. Gutierrez, F.J. Lazaro, A.J.
Fleming, T.G. St Pierre, M.R. Mikhael and P. Ponka
Poster #34
MITOCHONDRIAL PROTEIN, FRATAXIN, IS DOWNREGULATED IN HEMODIALYSIS PATIENTS
Takeshi Nakanishi, MD, PhD; Yukiko Hasuike, MD,PhD; Soshi Yorifuji, MD; Ayako Matsumoto, MD; Mana Yahiro, MD, PhD;
Aritoshi Kida, MD; Yasuyuki Nagasawa, MD, PhD; Takahiro Kuragano, MD, PhD
Poster #35
FRATAXIN IS A REGULATOR OF THE FE-S BIOGENESIS
Salvatore Adinolfi, biologist
Poster #39
MITOCHONDRIA-TARGETED IRON CHELATORS FOR THE MONITORING AND ADJUSTMENT OF CELLULAR LABILE
IRON POOLS
Vincenzo Abbate, PhD; Robert Hider, PhD; Charareh Pourzand, PhD; Olivier Reelfs, PhD
Poster #62
DOWN REGULATION OF RESPIRATORY ENZYMES IN FRATAXIN DEFICIENT YEAST IS MEDIATED BY THE
METABOLIC REGULATORS ADR1 AND CTH2
David Alsina, Armando Moreno-Cermeño, Elia Obis, Joaquim Ros and Jordi Tamarit
Poster #64
DEFINING THE ARCHITECTURE OF THE MITOCHODRIAL IRON-SULFUR CLUSTER ASSEMBLY MACHINERY
Belinda Galean
Poster #214
A CELL MODEL FOR FRIEDREICH ATAXIA USING DORSAL ROOT GANGLIA NEURONS
Stefka Mincheva-Tasheva, PhD, Elia Obis, Joaquim Ros, PhD and Jordi Tamarit, PhD
Poster #231
NOVEL LIGHT-ACTIVATED CAGED IRON CHELATORS: TARGETED PRODRUGS FOR IRON RELATED-DISORDERS
Benjamin Young, MPharm, Olivier Reelfs, PhD, DSc, Asma Aroun, PhD, Seyed Ali Miri, BSc, Magnus Hoffmann, BSc,
Charareh Pourzand, PhD, DSc and Ian Eggleston
Poster #273
A PRIMARY CULTURE OF CARDIOMYOCYTES WITH FRATAXIN DEFICIENCY EXHIBITS A METABOLIC SWITCH
BEFORE IRON DISARRANGEMENTS
Elia Obis, David Alsina, Joaquim Ros and Jordi Tamarit
Therapeutic Strategies in Friedreich's Ataxia
Therapeutic Strategies in Friedreich's Ataxia. Timothy E. Richardson, Heather N. Kelly, Amanda E. Yu, James W. Simpkins; Brain Research, Available online 13 April 2013. http://dx.doi.org/10.1016/j.brainres.2013.04.005
Keywords: Friedreich's ataxia (FA), FXN, frataxin, therapeutic research, ongoing treatment strategies, 17β-estradiol, methylene blue.
Keywords: Friedreich's ataxia (FA), FXN, frataxin, therapeutic research, ongoing treatment strategies, 17β-estradiol, methylene blue.
Thursday, April 11, 2013
Cardiomyopathy and the electrocardiogram in Friedreich's ataxia
Cardiomyopathy and the electrocardiogram in Friedreich's ataxia . Alexandra H Wood, Simon W Dubrey; British Journal of Hospital Medicine, Vol. 74, Iss. 4, 09 Apr 2013, pp 232 - 233
Iron uptake in quiescent and inflammation-activated astrocytes: A potentially neuroprotective control of iron burden
Iron uptake in quiescent and inflammation-activated astrocytes: A potentially neuroprotective control of iron burden. Ilaria Pelizzoni, Daniele Zacchetti, Alessandro Campanella, Fabio Grohovaz, Franca Codazzi; Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Available online 11 April 2013. http://dx.doi.org/10.1016/j.bbadis.2013.04.007
OPEN ACCESS
Keywords: Astrocytes, NTBI, iron uptake, DMT1, activation process, TRP channels, neuroinflammation
OPEN ACCESS
Keywords: Astrocytes, NTBI, iron uptake, DMT1, activation process, TRP channels, neuroinflammation
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