Idebenone and neuroprotection: antioxidant, pro-oxidant, or electron carrier?. Sausan Jaber, Brian M. Polster; Journal of Bioenergetics and Biomembranes
September 2014 DOI 10.1007/s10863-014-9571-y
Monday, September 29, 2014
Sunday, September 28, 2014
A Novel Mouse Model for Friedreich's Ataxia
A Novel Mouse Model for Friedreich's Ataxia. Inventors: Geschwind, Daniel H.; UCLA Office of Intellectual Property & Industry Sponsored Research
This new model exhibits various symptoms observed in FRDA patients, including cardiac atrophy, elevated iron-responsive proteins, neurodegeneration, motor neuropathy–and for the first time in an FRDA model–scoliosis and ataxia.
This new model exhibits various symptoms observed in FRDA patients, including cardiac atrophy, elevated iron-responsive proteins, neurodegeneration, motor neuropathy–and for the first time in an FRDA model–scoliosis and ataxia.
Next-Generation R&D Partnerships: The NCATS Success Story
Next-Generation R&D Partnerships: The NCATS Success Story , Information Technology and Innovation Foundation.
Free event on Wednesday, October 29, 2014,10:00 AM - 11:30 AM, Rayburn House Office Building 45 Independence Ave SW
(2168) Washington, DC20515
Free event on Wednesday, October 29, 2014,10:00 AM - 11:30 AM, Rayburn House Office Building 45 Independence Ave SW
(2168) Washington, DC20515
FGF8 Activates Proliferation and Migration in Mouse Post-Natal Oligodendrocyte Progenitor Cells
FGF8 Activates Proliferation and Migration in Mouse Post-Natal Oligodendrocyte Progenitor Cells . Pablo Cruz-Martinez, Almudena Martinez-Ferre, Jesus Jaramillo-Merchán, Alicia Estirado, Salvador Martinez, Jonathan Jones; PLoS ONE 9(9): e108241. doi:10.1371/journal.pone.0108241
Open Access, full text pdf
Open Access, full text pdf
Sunday, September 21, 2014
Understanding the frustration arising from the competition between function, misfolding, and aggregation in a globular protein.
Understanding the frustration arising from the competition between function, misfolding, and aggregation in a globular protein. Gianni S, Camilloni C, Giri R, Toto A, Bonetti D, Morrone A, Sormanni P, Brunori M, Vendruscolo M. Proc Natl Acad Sci U S A. 2014 Sep 16. pii: 201405233. [Epub ahead of print]
R2* mapping for brain iron: Associations with cognition in normal aging
R2* mapping for brain iron: Associations with cognition in normal aging; Christine Ghadery, Lukas Pirpamer, Edith Hofer, Christian Langkammer, Katja Petrovic, Marisa Loitfelder, Petra Schwingenschuh, Stephan Seiler, Marco Duering, Eric Jouvent, Helena Schmidt, Franz Fazekas, Jean-Francois Mangin, Hugues Chabriat, Martin Dichgans, Stefan Ropele, Reinhold Schmidt; Neurobiology of Aging, Available online 19 September 2014, ISSN 0197-4580, http://dx.doi.org/10.1016/j.neurobiolaging.2014.09.013.
We found the highest iron concentration in the globus pallidus, and pallidal as well as putaminal iron was significantly and inversely associated with cognitive performance in all cognitive domains, except memory. These associations were iron load-dependent. Vascular brain lesions and brain volume did not mediate the relationship between iron and cognitive performance.
We found the highest iron concentration in the globus pallidus, and pallidal as well as putaminal iron was significantly and inversely associated with cognitive performance in all cognitive domains, except memory. These associations were iron load-dependent. Vascular brain lesions and brain volume did not mediate the relationship between iron and cognitive performance.
Overlapping Binding Sites of the Frataxin Homologue Assembly Factor and the Heat Shock Protein 70 Transfer Factor on the Isu Iron-sulfur Cluster Scaffold Protein
Overlapping Binding Sites of the Frataxin Homologue Assembly Factor and the Heat Shock Protein 70 Transfer Factor on the Isu Iron-sulfur Cluster Scaffold Protein . Mateusz Manicki, Julia Majewska, Szymon Ciesielski, Brenda Schilke, Anna Blenska, Jacek Kominek, Jaroslaw Marszalek, Elizabeth A. Craig, and Rafal Dutkiewicz; J. Biol. Chem. jbc.M114.596726. First Published on September 16, 2014, doi:10.1074/jbc.M114.596726
FULL TEXT PDF
FULL TEXT PDF
Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery
Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery. Nunziata Maio, Tracey.A. Rouault; Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Available online 19 September 2014, ISSN 0167-4889, http://dx.doi.org/10.1016/j.bbamcr.2014.09.009.
The tissue- specific phenotypes of FRDA might arise from the critical requirement of specific isoforms of FXN, which may be severely decreased in the disease. Interestingly, two rare frataxin isoforms have been recently identified [198], and their expression was substantially reduced in the heart (isoform III), and cerebellum (isoform II) of a FRDA patient, suggesting that novel expression patterns may explain some FRDA tissue specific phenotypes.
The tissue- specific phenotypes of FRDA might arise from the critical requirement of specific isoforms of FXN, which may be severely decreased in the disease. Interestingly, two rare frataxin isoforms have been recently identified [198], and their expression was substantially reduced in the heart (isoform III), and cerebellum (isoform II) of a FRDA patient, suggesting that novel expression patterns may explain some FRDA tissue specific phenotypes.
Tuesday, September 16, 2014
The role of iron in brain ageing and neurodegenerative disorders
The role of iron in brain ageing and neurodegenerative disorders. Roberta J Ward, Fabio A Zucca, Jeff H Duyn, Robert R Crichton, Luigi Zecca; The Lancet Neurology, Volume 13, Issue 10, October 2014, Pages 1045-1060, ISSN 1474-4422, http://dx.doi.org/10.1016/S1474-4422(14)70117-6.
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