Project: Investigating the neuroprotective and neuroregenerative properties of bone marrow stem cell mobilising drugs in Friedreich ataxia.

Project: Investigating the neuroprotective and neuroregenerative properties of bone marrow stem cell mobilising drugs in Friedreich ataxia.
Alastair Wilkins (Principal Investigator)
Neil Scolding (Co-Investigator)
Funded Period: may 12 - abr 15
University of Bristol, Bristol, United Kingdom, Brunel University, Uxbridge, United Kingdom

Mössbauer Study and Modeling of Iron Import and Trafficking in Human Jurkat Cells

Mössbauer Study and Modeling of Iron Import and Trafficking in Human Jurkat Cells. Nema D. Jhurry, Mrinmoy Chakrabarti, Sean P. McCormick, Vishal M. Gohil, and Paul A. Lindahl; Biochemistry, Article ASAP. DOI: 10.1021/bi401015t Publication Date (Web): November 1, 2013.

Clinical features and progression of cardiomyopathy in patients with Friedreich's ataxia

Clinical features and progression of cardiomyopathy in patients with Friedreich's ataxia. Vincent Pureza, Linda Hasadsri, W. Edward Highsmith, Devin Oglesbee; Mitochondrion, Volume 13, Issue 6, November 2013, Page 925.

"We hypothesize that the cardiac manifestations will predominantly present as a diastolic dysfunction"

Iron metabolism and the role of iron in neurodegenerative disorders

Iron metabolism and the role of iron in neurodegenerative disorders; Maya Hadzhieva, Elmar Kirches, Christian Mawrin. Neuropathology and Applied Neurobiology
Accepted Article (Accepted, unedited articles published online and citable. The final edited and typeset version of record will appear in future.) DOI: 10.1111/nan.12096

KEYWORDS: neurodegeneration; ROS; iron; mitoferrin; TfR; DMT1; ferritin; frataxin; IscU

Late onset Friedreich ataxia: clinical description of a family in Argentina.

Late onset Friedreich ataxia: clinical description of a family in Argentina.; Perez Akly M and Alvarez F Medicina (B Aires). 2013;73(5):457-460.

FULL TEXT PDF (Spanish)

Unclassified cardiomyopathies in neuromuscular disorders.

Unclassified cardiomyopathies in neuromuscular disorders.. Finsterer J, Stöllberger; Wien Med Wochenschr 2013 Oct 24

Clinical Neurogenetics : Friedreich Ataxia

Clinical Neurogenetics : Friedreich Ataxia. Abigail Collins; Neurologic Clinics, Volume 31, Issue 4, November 2013, Pages 1095-1120. http://dx.doi.org/10.1016/j.ncl.2013.05.002

Modelling biochemical features of mitochondrial neuropathology

Modelling biochemical features of mitochondrial neuropathology ; Matthew J. Bird, David R. Thorburn, Ann E. Frazier; Biochimica et Biophysica Acta (BBA) - General Subjects, Available online 23 October 2013. http://dx.doi.org/10.1016/j.bbagen.2013.10.017

Keywords: Neuropathology; mitochondrial disease; mouse models; cybrids; iPS; OXPHOS; membrane potential; Ca2 +; reactive oxygen species; mitochondrial morphology; frataxin

The Nervous System Cytoskeleton under Oxidative Stress

The Nervous System Cytoskeleton under Oxidative Stress. Gardiner, J.; Overall, R.; Marc, J.; Diseases 2013, 1, 36-50.

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Fixing Frataxin: “Ironing Out” the Metabolic Defect in Friedreich's Ataxia

Fixing Frataxin: “Ironing Out” the Metabolic Defect in Friedreich's Ataxia. A. Anzovino, D. J. R. Lane, M. L.-H. Huang, D. R. Richardson, British Journal of Pharmacology, Accepted manuscript online: 21 OCT 2013, DOI: 10.1111/bph.12470

Keywords: Frataxin; Mitochondrial Iron Accumulation; Oxidative Stress; Autophagy; Cardio- and Neuro-Degeneration; Friedriech's Ataxia