Nature Neuroscience 14, 429–436, (2011) doi:10.1038/nn.2762
Published online, 20 March 2011.
Claire Jacob, Carlos N Christen, Jorge A Pereira, Christian Somandin, Arianna Baggiolini, Pirmin Lötscher, Murat Özçelik, Nicolas Tricaud, Dies Meijer, Teppei Yamaguchi, Patrick Matthias & Ueli Suter
In Schwann cells, HDAC1 and HDAC2 exert specific primary functions: HDAC2 activates the transcriptional program of myelination in synergy with Sox10, whereas HDAC1 controls Schwann cell survival.
Monday, March 28, 2011
Sunday, March 27, 2011
Stem Cell Research May Lead To New Treatments For Parkinson's Disease, Huntington's Disease, Multiple Sclerosis, Stroke, Spinal Cord Injury
Medical News Today, Article Date: 26 Mar 2011
As always, they forget the rare diseases, but I think this aproach is also useful for FA.
A group of scientists at Marshall University is conducting research that may someday lead to new treatments for repair of the central nervous system.
Price said the neurons they found appear to have many of the qualities desired for cells being used in development of therapies for slowly progressing, degenerative conditions
Multipotent progenitor cells derived from adult peripheral blood of swine have high neurogenic potential in vitro. Nadja Spitzer, Gregory S. Sammons, Heather M. Butts, Lawrence M. Grover, Elmer M. Price.Journal of Cellular Physiology, 2011; DOI: 10.1002/jcp.22670
As always, they forget the rare diseases, but I think this aproach is also useful for FA.
A group of scientists at Marshall University is conducting research that may someday lead to new treatments for repair of the central nervous system.
Price said the neurons they found appear to have many of the qualities desired for cells being used in development of therapies for slowly progressing, degenerative conditions
Multipotent progenitor cells derived from adult peripheral blood of swine have high neurogenic potential in vitro. Nadja Spitzer, Gregory S. Sammons, Heather M. Butts, Lawrence M. Grover, Elmer M. Price.Journal of Cellular Physiology, 2011; DOI: 10.1002/jcp.22670
Thursday, March 24, 2011
Short-term low-frequency electrical stimulation enhanced remyelination of injured peripheral nerves by inducing the promyelination effect of brain-derived neurotrophic factor on Schwann cell polarization
J Neurosci Res. 2010 Sep;88(12):2578-87.
Wan L, Zhang S, Xia R, Ding W.
Department of Anatomy, Medical College of Nanchang University, Nanchang 330006, China.
Keywords: Electrical stimulation (ES), nerve injuries, direct neurite outgrowth, peripheral remyelination, P0, Par-3, brain-derived neurotrophic factor (BDNF), dorsal root ganglion neuron/Schwann cell, 20-Hz ES, Schwann cell myelination.
Wan L, Zhang S, Xia R, Ding W.
Department of Anatomy, Medical College of Nanchang University, Nanchang 330006, China.
Keywords: Electrical stimulation (ES), nerve injuries, direct neurite outgrowth, peripheral remyelination, P0, Par-3, brain-derived neurotrophic factor (BDNF), dorsal root ganglion neuron/Schwann cell, 20-Hz ES, Schwann cell myelination.
Wednesday, March 16, 2011
Surgical treatment of neurological scoliosis using hybrid construct (lumbar transpedicular screws plus thoracic sublaminar acrylic loops).
Eur Spine J. 2011 Mar 15
La Rosa G, Giglio G, Oggiano L.
Department of Paediatric Surgery, Orthopaedic Unit, Research Institute Paediatric Hospital Bambino Gesù Palidoro, Fiumicino, Italy.
Keywords: segmental spine instrumentation, Cotrel-Dobousset (CD), sublaminar acrylic loops (Universal Clamp), spinal fixation implant, neurologic scoliosis, Socore TM spinal assembly, transpedicular lumbar screws, thoracic hooks, cerebral palsy, Friedreich ataxia, Aicardi syndrome, short operative time, low vascular and neurologic risks.
La Rosa G, Giglio G, Oggiano L.
Department of Paediatric Surgery, Orthopaedic Unit, Research Institute Paediatric Hospital Bambino Gesù Palidoro, Fiumicino, Italy.
Keywords: segmental spine instrumentation, Cotrel-Dobousset (CD), sublaminar acrylic loops (Universal Clamp), spinal fixation implant, neurologic scoliosis, Socore TM spinal assembly, transpedicular lumbar screws, thoracic hooks, cerebral palsy, Friedreich ataxia, Aicardi syndrome, short operative time, low vascular and neurologic risks.
Cotrel-Dubousset instrumentation in neuromuscular scoliosis.
Eur Spine J. 2011 Mar 15.
Piazzolla A, Solarino G, De Giorgi S, Mori CM, Moretti L, De Giorgi G.
Dipartimento di Metodologia Clinica e Tecnologie Medico Chirurgiche, Università degli Studi di Bari, Bari, Italy.
Keywords: retrospective study, neuromuscular scoliosis (NMS), Spinal deformity, brace, surgery, solid spinal fusion, Cotrel-Dubousset (CD) surgical technique, Friedreich's ataxia, cerebral palsy, muscular dystrophy, polio, syringomyelia, spinal atrophy, kyphosis, lordosis.
Piazzolla A, Solarino G, De Giorgi S, Mori CM, Moretti L, De Giorgi G.
Dipartimento di Metodologia Clinica e Tecnologie Medico Chirurgiche, Università degli Studi di Bari, Bari, Italy.
Keywords: retrospective study, neuromuscular scoliosis (NMS), Spinal deformity, brace, surgery, solid spinal fusion, Cotrel-Dubousset (CD) surgical technique, Friedreich's ataxia, cerebral palsy, muscular dystrophy, polio, syringomyelia, spinal atrophy, kyphosis, lordosis.
Tuesday, March 15, 2011
Protein That Keeps Brain Signals Intact Yields Clue To Disorders
Medical News Today, Article Date: 14 Mar 2011
Researchers at the University of Edinburgh in the UK have found a new clue for understanding neurological disorders that occur when the brain can't send signals to other parts of the body.
Original paper:
"A Critical Role for Neurofascin in Regulating Action Potential Initiation through Maintenance of the Axon Initial Segment."
Barbara Zonta, Anne Desmazieres, Arianna Rinaldi, Steven Tait, Diane L. Sherman, Matthew F. Nolan, Peter J. Brophy.
Neuron, 10 March 2011 (Vol. 69, Issue 5, pp. 945-956).
DOI:10.1016/j.neuron.2011.02.021
FULL TEXT PDF
Researchers at the University of Edinburgh in the UK have found a new clue for understanding neurological disorders that occur when the brain can't send signals to other parts of the body.
Original paper:
"A Critical Role for Neurofascin in Regulating Action Potential Initiation through Maintenance of the Axon Initial Segment."
Barbara Zonta, Anne Desmazieres, Arianna Rinaldi, Steven Tait, Diane L. Sherman, Matthew F. Nolan, Peter J. Brophy.
Neuron, 10 March 2011 (Vol. 69, Issue 5, pp. 945-956).
DOI:10.1016/j.neuron.2011.02.021
FULL TEXT PDF
Monday, March 14, 2011
Mitochondria-Targeted Small Molecule Therapeutics and Probes
Antioxidants & Redox Signaling. doi:10.1089/ars.2011.3969.
Robin A Smith, Dunedin, New Zealand; Richard C Hartley, Glasgow, United Kingdom; MIke Murphy, MRC, Mitochondrial Biology Unit, Cambridge, United Kingdom
Robin A Smith, Dunedin, New Zealand; Richard C Hartley, Glasgow, United Kingdom; MIke Murphy, MRC, Mitochondrial Biology Unit, Cambridge, United Kingdom
Saturday, March 12, 2011
Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA).
Am Heart J. 2011 Mar;161(3):639-645.e1. Epub 2011 Jan 31.
Lagedrost SJ, Sutton MS, Cohen MS, Satou GM, Kaufman BD, Perlman SL, Rummey C, Meier T, Lynch DR.
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA; Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA; The Children's Hospital of Philadelphia, Philadelphia, PA.
CONCLUSIONS: The study does not provide evidence of benefit in this cohort over a 6-month treatment period.
Lagedrost SJ, Sutton MS, Cohen MS, Satou GM, Kaufman BD, Perlman SL, Rummey C, Meier T, Lynch DR.
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA; Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA; The Children's Hospital of Philadelphia, Philadelphia, PA.
CONCLUSIONS: The study does not provide evidence of benefit in this cohort over a 6-month treatment period.
A Combined Nucleic Acid and Protein Analysis in Friedreich Ataxia: Implications for Diagnosis, Pathogenesis and Clinical Trial Design
PLoS ONE 6(3): e17627. doi:10.1371/journal.pone.0017627
Francesco Saccà1*, Giorgia Puorro1, Antonella Antenora1, Angela Marsili1, Alessandra Denaro1, Raffaele Piro1, Pierpaolo Sorrentino1, Chiara Pane1, Alessandra Tessa2, Vincenzo Brescia Morra1, Sergio Cocozza3, Giuseppe De Michele1, Filippo M. Santorelli2, Alessandro Filla1
1 Department of Neurological Sciences, University Federico II, Naples, Italy, 2 Molecular Medicine, IRCCS Stella Maris, Pisa, Italy, 3 Department of Cellular and Molecular Biology, University Federico II, Naples, Italy
OPEN ACCESS
Methodology/Principal Findings
We enrolled 24 patients with classic FRDA phenotype (cFA), 6 late onset FRDA (LOFA), all homozygous for GAA expansion, 5 pFA cases who harbored the GAA expansion in compound heterozygosis with FXN point mutations (namely, p.I154F, c.482+3delA, p.R165P), 33 healthy expansion carriers, and 29 healthy controls. DNA was genotyped for GAA expansion, mRNA/FXN was quantified in real-time, and frataxin protein was measured using lateral-flow immunoassay in peripheral blood mononuclear cells (PBMCs). Mean residual levels of frataxin, compared to controls, were 35.8%, 65.6%, 33%, and 68.7% in cFA, LOFA, pFA and healthy carriers, respectively. Comparison of both cFA and pFA with controls resulted in 100% sensitivity and specificity, but there was overlap between LOFA, carriers and controls. Frataxin levels correlated inversely with GAA1 and GAA2 expansions, and directly with age at onset. Messenger RNA expression was reduced to 19.4% in cFA, 50.4% in LOFA, 52.7% in pFA, 53.0% in carriers, as compared to controls (p<0.0001). mRNA levels proved to be diagnostic when comparing cFA with controls resulting in 100% sensitivity and specificity. In cFA and LOFA patients mRNA levels correlated directly with protein levels and age at onset, and inversely with GAA1 and GAA2.
FULL TEXT PDF
Francesco Saccà1*, Giorgia Puorro1, Antonella Antenora1, Angela Marsili1, Alessandra Denaro1, Raffaele Piro1, Pierpaolo Sorrentino1, Chiara Pane1, Alessandra Tessa2, Vincenzo Brescia Morra1, Sergio Cocozza3, Giuseppe De Michele1, Filippo M. Santorelli2, Alessandro Filla1
1 Department of Neurological Sciences, University Federico II, Naples, Italy, 2 Molecular Medicine, IRCCS Stella Maris, Pisa, Italy, 3 Department of Cellular and Molecular Biology, University Federico II, Naples, Italy
OPEN ACCESS
Methodology/Principal Findings
We enrolled 24 patients with classic FRDA phenotype (cFA), 6 late onset FRDA (LOFA), all homozygous for GAA expansion, 5 pFA cases who harbored the GAA expansion in compound heterozygosis with FXN point mutations (namely, p.I154F, c.482+3delA, p.R165P), 33 healthy expansion carriers, and 29 healthy controls. DNA was genotyped for GAA expansion, mRNA/FXN was quantified in real-time, and frataxin protein was measured using lateral-flow immunoassay in peripheral blood mononuclear cells (PBMCs). Mean residual levels of frataxin, compared to controls, were 35.8%, 65.6%, 33%, and 68.7% in cFA, LOFA, pFA and healthy carriers, respectively. Comparison of both cFA and pFA with controls resulted in 100% sensitivity and specificity, but there was overlap between LOFA, carriers and controls. Frataxin levels correlated inversely with GAA1 and GAA2 expansions, and directly with age at onset. Messenger RNA expression was reduced to 19.4% in cFA, 50.4% in LOFA, 52.7% in pFA, 53.0% in carriers, as compared to controls (p<0.0001). mRNA levels proved to be diagnostic when comparing cFA with controls resulting in 100% sensitivity and specificity. In cFA and LOFA patients mRNA levels correlated directly with protein levels and age at onset, and inversely with GAA1 and GAA2.
FULL TEXT PDF
Friday, March 11, 2011
Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model
Neurobiology of Disease, doi:10.1016/j.nbd.2011.02.016
Chiranjeevi Sandi a, Ricardo Mouro Pinto a, 1, Sahar Al-Mahdawi a, Vahid Ezzatizadeh a, Glenn Barnes b, Steve Jones b, James R. Rusche b, Joel M. Gottesfeld c and Mark A. Pook a
a Division of Biosciences, School of Health Sciences and Social Care, Brunel University, Uxbridge, UB8 3PH, UK
b Repligen Corporation, Waltham, MA, USA
c Department of Molecular Biology, The Scripps Research Institute, La Jolla, CA, USA
Available online 10 March 2011.
keywords: Friedreich ataxia (FRDA), Histone deacetylase (HDAC) inhibitors, pimelic o-aminobenzamide compounds 106, 109 and 136, FXN gene silencing, short-term studies, long-term therapeutic effects, FRDA mouse model.
Chiranjeevi Sandi a, Ricardo Mouro Pinto a, 1, Sahar Al-Mahdawi a, Vahid Ezzatizadeh a, Glenn Barnes b, Steve Jones b, James R. Rusche b, Joel M. Gottesfeld c and Mark A. Pook a
a Division of Biosciences, School of Health Sciences and Social Care, Brunel University, Uxbridge, UB8 3PH, UK
b Repligen Corporation, Waltham, MA, USA
c Department of Molecular Biology, The Scripps Research Institute, La Jolla, CA, USA
Available online 10 March 2011.
keywords: Friedreich ataxia (FRDA), Histone deacetylase (HDAC) inhibitors, pimelic o-aminobenzamide compounds 106, 109 and 136, FXN gene silencing, short-term studies, long-term therapeutic effects, FRDA mouse model.
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