Magnetic Resonance Imaging and Spectroscopy of the Central Nervous System in Friedreich's Ataxia

Magnetic Resonance Imaging and Spectroscopy of the Central Nervous System in Friedreich's Ataxia.

University of Minnesota - Clinical and Translational Science Institute

ClinicalTrials.gov Identifier: NCT01776164
First received: January 14, 2013
Last updated: January 22, 2013
Last verified: January 2013



.../... the investigators aim at utilizing the capabilities of Magnetic Resonance Imaging (MRI) and Spectroscopy (MRS). Using techniques called Diffusion Imaging, resting-state functional MRI, and Proton Spectroscopy (1H MRS), the investigators propose to determine the differences in the connectivity and neurochemistry of the spinal cord and the brain .../...

Glial Cells Assist in the Repair of Injured Nerves

Glial Cells Assist in the Repair of Injured Nerves. Max-Planck-Gesellschaft. ScienceDaily, 28 Jan. 2013. Web. 29 Jan. 2013.

Jan. 28, 2013 — Unlike the brain and spinal cord, the peripheral nervous system has an astonishing capacity for regeneration following injury. Researchers at the Max Planck Institute of Experimental Medicine in Göttingen have discovered that, following nerve damage, peripheral glial cells produce the growth factor neuregulin1, which makes an important contribution to the regeneration of damaged nerves.

Original source (Journal reference): A role for Schwann cell-derived neuregulin-1 in remyelination. Stassart RM, Fledrich R, Velanac V, Brinkmann BG, Schwab MH, Meijer D, Sereda MW, Nave KA. Nature Neuroscience, 2012; 16 (1): 48 DOI: 10.1038/nn.3281

Eye movements in patients with neurodegenerative disorders

Eye movements in patients with neurodegenerative disorders. Review, Tim J. Anderson & Michael R. MacAskill, Nature Reviews Neurology, advance online publication, Published online 22 January 2013 | doi:10.1038/nrneurol.2012.273

Keywords: neural pathways, brain regions, eye movements, cerebrum, brainstem, cerebellum, neurodegeneration, neurodegenerative disorders, disease severity, objective evaluation, saccade performance.

Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich’s ataxia YG8R mouse model

Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich’s ataxia YG8R mouse model . Dr. Yuxi Shan, Dr. Robert A Schoenfeld, Genki Hayashi, Dr. Eleonora Napoli, Dr. Tasuku Akiyama, Dr. Mirela Iodi-Carstens, Dr. Earl E. Carstens, Dr. Mark A. Pook, and Dr. Gino Cortopassi. Antioxidants & Redox Signaling. -Not available-, ahead of print. doi:10.1089/ars.2012.4537.

"Frataxin expression was significantly correlated with Nrf2 expression"

Keywords: Oxidative stress, Dorsal Root Ganglion (DRG) neurons, transcripts encoding antioxidants, peroxiredoxins, glutaredoxins, glutathione-S-transferase, antioxidant transcription factor Nrf2, frataxin, thioredoxin reductase.

Mitochondria in cardiac hypertrophy and heart failure

Mitochondria in cardiac hypertrophy and heart failure. Mariana G. Rosca, Bernard Tandler, Charles L. Hoppel. Journal of Molecular and Cellular Cardiology, Volume 55, February 2013, Pages 31-41.

Keywords: Heart failure, Cardiac hypertrophy, Mitochondria, Oxidative phosphorylation

Transition metals and mitochondrial metabolism in the heart

Transition metals and mitochondrial metabolism in the heart. Amy K. Rines, Hossein Ardehali, Journal of Molecular and Cellular Cardiology, Volume 55, February 2013, Pages 50-57. http://dx.doi.org/10.1016/j.yjmcc.2012.05.014

Highlights: "FRDA and doxorubicin toxicities are highlighted as disorders of iron deregulation.", "the heart is one of the most mitochondrially-rich tissues in the body"

The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization

The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization . Christopher A. G. Soderberg, Sreekanth Rajan, Aleksander V. Shkumatov, Oleksandr Gakh, Susanne Schaefer, Eva-Christina Ahlgren, Dmitri I. Svergun, Grazia Isaya and Salam Al-Karadaghi. J. Biol. Chem. jbc.M112.442285. First Published on January 23, 2013, doi:10.1074/jbc.M112.442285

Keywords: Iron-induced oligomerization, frataxin, catalyzed ferroxidation, iron availability, iron delivery.

The role of mitochondrial OXPHOS dysfunction in the development of neurologic diseases

The role of mitochondrial OXPHOS dysfunction in the development of neurologic diseases.M.E. Breuer, W.J. Koopman, S. Koene, M. Nooteboom, R.J. Rodenburg, P.H. Willems, J.A.M. Smeitink. Neurobiology of Disease, Volume 51, March 2013, Pages 27-34.

Keywords: Mitochondrial dysfunction, Neurodegenerative diseases, Bioenergetics, Cellular metabolism

Friedreich Ataxia: Neuropathology Revised.

Friedreich Ataxia: Neuropathology Revised.. Koeppen, Arnulf H. MD.; Mazurkiewicz, Jose. Journal of Neuropathology & Experimental Neurology, 72:2 2013 Feb pg 78-90.

Keywords: Friedreich ataxia, Low frataxin levels, iron-sulfur clusters, aconitase, dorsal root ganglia, dorsal columns, transsynaptic atrophy, Clarke column, dorsal spinocerebellar fibers, gracile and cuneate nuclei, sensory nerves, dentate nucleus.

Neuromuscular scoliosis

Neuromuscular scoliosis. R. Vialle, C. Thévenin-Lemoine, P. Mary; Orthopaedics & Traumatology: Surgery & Research, Available online 18 January 2013. DOI: http://dx.doi.org/10.1016/j.otsr.2012.11.002,


Highlight: "Surgical treatment of neuromuscular scoliosis differs from idiopathic scoliosis".
"Non-surgical measures rarely fully control progressive scoliosis".

Keywords: Neuromuscular scoliosis, surgical treatment, idiopathic scoliosis, complications rate, Non-surgical measures, muscular hypotonia.