Seminar at SWOSU: “The Role of Frataxin in the Neurodegenerative Disease Friedreich’s Ataxia”

Seminar at SWOSU: “The Role of Frataxin in the Neurodegenerative Disease Friedreich’s Ataxia”.
January 10, 2013, Dr. Laura Busenlehner, assistant professor in the Department of Chemistry at the University of Alabama, will give a seminar at Southwestern Oklahoma State University on Thursday, January 17, on the Weatherford campus.

Busenlehner’s seminar is titled “The Role of Frataxin in the Neurodegenerative Disease Friedreich’s Ataxia”. Her seminar will focus on the inorganic chemistry, biochemistry and biophysical characterization of the Frataxin protein.

STATegics, Inc. Announces a New Grant from Friedreich’s Ataxia Research Alliance

STATegics, Inc. Announces a New Grant from Friedreich’s Ataxia Research Alliance. PRLog (Press Release) - Jan. 8, 2013 - Menlo Park, California.

STATegics, Inc. awarded a new grant from the Friedreich’s Ataxia Research Alliance (FARA) to advance erythropoietin receptor agonist compounds for the treatment of Friedreich’s ataxia (FRDA).

Edison Pharmaceuticals Announces Initiation of EPI-743 Phase 2B Friedreich's Ataxia Clinical Trial

Edison Pharmaceuticals Announces Initiation of EPI-743 Phase 2B Friedreich's Ataxia Clinical Trial. PR Newswire, Press release Jan. 7, 2013, 12:01 a.m. EST

MOUNTAIN VIEW, Calif., Jan. 7, 2013 /PRNewswire via COMTEX/ -- Edison Pharmaceuticals today announced the initiation of a phase 2B study entitled, "Safety and Efficacy Study of EPI-743 on Visual Function in Patients with Friedreich's Ataxia." The trial is a placebo-controlled study lasting six months, followed by an extension phase in which all subjects will receive EPI-743.

More information on study specifics is available on ClinicalTrials.gov.

Somatosensory Temporal Discrimination Threshold Is Increased in Patients with Cerebellar Atrophy

Somatosensory Temporal Discrimination Threshold Is Increased in Patients with Cerebellar Atrophy. Fiore Manganelli, Raffaele Dubbioso, Chiara Pisciotta,
Antonella Antenora, Maria Nolano, Giuseppe De Michele, Alessandro Filla, Alfredo Berardelli, Lucio Santoro. The Cerebellum, January 2013. DOI 10.1007/s12311-012-0435-x

Keywords: millisecond range, cerebellar function, somatosensory temporal discrimination threshold testing.

NeuroDNet - an open source platform for constructing and analyzing neurodegenerative disease networks

NeuroDNet - an open source platform for constructing and analyzing neurodegenerative disease networks. Suhas V Vasaikar, Aditya K Padhi, Bhyravabhotla Jayaram and James Gomes. BMC Neuroscience 2013, 14:3 doi:10.1186/1471-2202-14-3, Published: 3 January 2013

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It is in this light that we have created a database, NeuroDNet, that includes information about twelve neurodegenerative diseases - adrenomyeloneuropathy, Alzheimer disease, amyotrophic lateral sclerosis, ataxia-telangiectasia, dentatorubral-pallidoluysian atrophy, Friedreich ataxia, frontotemporal dementia, Huntington disease, Lewy body dementia, Parkinson disease, prion disease, progressive supranuclear palsy. It accounts for the interactions and regulation between signaling molecules, genes and proteins. This database is also the first of its kind, which enables the construction and analysis of NDDs through PPI, regulatory and Boolean networks. We also present the results of three case studies, which demonstrate the power of the analytical tools featured in NeuroDNet.

In Epigenomics, Location Is Everything: Researchers Exploit Gene Position to Test 'Histone Code'

In Epigenomics, Location Is Everything: Researchers Exploit Gene Position to Test 'Histone Code'. Menzies Chen, Katherine Licon, Rei Otsuka, Lorraine Pillussend, Trey Idekersend. Cell Reports, 03 January 2013

Also: Science News, Jan. 3, 2013,
Jan. 3, 2013 — In a novel use of gene knockout technology, researchers at the University of California, San Diego School of Medicine tested the same gene inserted into 90 different locations in a yeast chromosome -- and discovered that while the inserted gene never altered its surrounding chromatin landscape, differences in that immediate landscape measurably affected gene activity.

RNA-mediated toxicity in neurodegenerative disease

RNA-mediated toxicity in neurodegenerative disease. Veronique V. Belzil, Tania F. Gendron, Leonard Petrucelli. Molecular and Cellular Neuroscience, Available online 29 December 2012

Keywords: RNA processing, bidirectional transcription, RAN translation, RNA foci, neurodegenerative diseases, mouse models, non-coding repeat expansions.

ViroPharma is set to initiate VP20629 Phase II trial in the second half of 2013

ViroPharma is set to initiate VP20629 Phase II trial in the second half of 2013

ViroPharma: Strengthening Financial Profile & Pipeline Indicate Long-Term Upside, Ivan Deryugin, December 27, 2012

VP20629 is licensed from Intellect Neurosciences for the treatment of Friedreich’s Ataxia (FA), a rare neurodegenerative disease that affects about 1 in 50,000 people. Symptoms of FA include heart disease, speech problems, and gait disturbance. It is estimated that there are 6,000 FA patients here in the United States. Phase I data for VP20629 showed that the drug was well-tolerated at all dose levels, and ViroPharma is set to initiate a Phase II trial in the second half of 2013, and the company plans to file for orphan drug status once Phase II data is available

About VP 20629

Missense Mutations Linked to Friedreich Ataxia Have Different but Synergistic Effects on Mitochondrial Frataxin Isoforms

Missense Mutations Linked to Friedreich Ataxia Have Different but Synergistic Effects on Mitochondrial Frataxin Isoforms . Hongqiao Li, Oleksandr Gakh, Douglas Y. Smith IV, Wasantha K Ranatunga and Grazia Isaya. J. Biol. Chem. jbc.M112.435263. First Published on December 26, 2012, doi:10.1074/jbc.M112.435263

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Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly

Mammalian frataxin controls sulfur production and iron entry during de novo Fe4S4 cluster assembly. Florent Colin , Alain Martelli , Martin Clémancey , Jean-Marc Latour , Serge Gambarelli , Laura Zeppieri , Catherine Birck , Adeline Page , Hélène Puccio , and Sandrine Ollagnier de Choudens. J. Am. Chem. Soc., Just Accepted Manuscript, DOI: 10.1021/ja308736e. Publication Date (Web): December 22, 2012

Keywords: Fe-S clusters, cysteine desulfurase complex (NFS1/ISD11), frataxin (FXN), Freidreich’s ataxia, cysteine desulfurization, aconitase (mACO2).