Friday, October 8, 2010

Evaluation of neuropsychological functions in patients with Friedreich ataxia before and after cognitive therapy

Funct Neurol. 2010 Apr-Jun;25(2):81-5.
Ciancarelli I, Cofini V, Carolei A.

Keywords: Friedreich ataxia (FA, severe disability, cognitive functions, mood disorders, conflicting results, role of cognitive rehabilitation therapy, MMSE, Rey 15-item Memorization Test, Raven's Colored Progressive Matrices, the Phonemic Verbal Fluency Test, the Symbol Digit Modalities Test, Zung scale.

Wednesday, October 6, 2010

Remarkably Efficient New Method For Generating Human Stem Cells

Medical News Today, Article Date: 02 Oct 2010 - 0:00 PDT

The ability to efficiently generate patient-specific stem cells from differentiated cells and then reliably direct them to form specialized cells (like neurons or muscle) has tremendous therapeutic potential for replacing diseased or damaged tissues... read more


Technology Review, Friday, October 1, 2010

A New Way to Make Stem Cells

Using RNA instead of DNA could avoid the health risks--and the political pitfalls--of stem-cell treatments.

A Harvard researcher has developed a way to make pluripotent stem cells that solves several of the major impediments to using them to treat human diseases. read more...

Tuesday, October 5, 2010

Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly.

J Biol Chem. 2010 Oct 2. [Epub ahead of print]

Gakh O, Bedekovics T, Duncan SF, Smith DY 4th, Berkholz DS, Isaya G.
Mayo Clinic, United States.

Keywords: frataxin (FXN), Fe-S cluster, FXN mRNA, FXN42-210, FXN81-210, levels of both isoforms are relevant, FXN81-210/FXN42-210 molar ratio, replacement therapies.

Saturday, October 2, 2010

PGC-1 Coactivators in Cardiac Development and Disease

Circulation Research. 2010;107:825-838
doi: 10.1161/CIRCRESAHA.110.223818

Daniel P. Kelly, Guest Editor
Glenn C. Rowe, Aihua Jiang, Zolt Arany

From the Cardiovascular Institute at the Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Mass.

Key Words: PGC-1 • metabolism • heart failure • mitochondria

Thursday, September 30, 2010

Progress and prospects: stem cells and neurological diseases

Gene Therapy , (30 September 2010) | doi:10.1038/gt.2010.130

S Gögel, M Gubernator and S L Minger

Review.

Keywords: central nervous system, degenerative neurological conditions, Parkinson's disease (PD), Alzheimer's disease, Huntington's disease (HD), cerebrovascular damage (for example, stroke), spinal cord injury, transplantation of stem cells, stem cell-derived progenitors, induced pluripotent stem cells.

Tuesday, September 28, 2010

Spinal convergence of motor and sensory pathways

Nature Neuroscience 13, 1160 (2010)
Published online: 27 September 2010 | doi:10.1038/nn1010-1160

Min Cho

KEYWORDS: motor execution, proprioceptive sensory feedback, motor command.

Monday, September 27, 2010

Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex

Biochemistry, Just Accepted Manuscript
Publication Date (Web): September 27, 2010

Chi-Lin Tsai and David P. Barondeau, Department of Chemistry, Texas A&M
University, College Station, TX 77842, USA.

Full text pdf

Scientists Discover Gene That Controls Stem Cells In Central Nervous System

Medical News Today, 27 Sep 2010

"With the knowledge that the gene Sox9 plays a central role in the development of our nervous system, we are one step closer to being able to control stem cells in the brain and regenerate different kinds of nerve cells." ....read more....

Sunday, September 26, 2010

Restless Legs and Substantia Nigra Hypoechogenicity are Common Features in Friedreich's Ataxia.

Cerebellum. 2010 Sep 24.

Synofzik M, Godau J, Lindig T, Schöls L, Berg D.

Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, Tübingen, Germany.

KEYWORDS: Friedreich's ataxia (FA), restless legs syndrome (RLS), multisystemic network dysfunction, peripheral neuropathy, disturbances in subcellular brain iron homeostasis.

Combined Therapy with Idebenone and Deferiprone in Patients with Friedreich's Ataxia.

Cerebellum. 2010 Sep 24.

Velasco-Sánchez D, Aracil A, Montero R, Mas A, Jiménez L, O'Callaghan M, Tondo M, Capdevila A, Blanch J, Artuch R, Pineda M.

Cardiology Department, Sant Joan de Déu Hospital and the University of Barcelona, 2, Passeig Sant Joan de Déu, 08950 Esplugues, Barcelona, Spain

KEYWORDS: Iron chelators,Friedreich's ataxia, mitochondrial iron pools, neurological signs, cardiac function parameters, prospective open-label single-arm study, idebenone (20 mg/kg/day), deferiprone (20 mg/kg/day), International Cooperative Ataxia Rating Scale (ICARS) scores, echocardiographic measurements, MRI (magnetic resonance imaging) techniques, neutropenia, progressive reduction of plasma iron parameters.