The Friedreich’s Ataxia protein frataxin modulates DNA base excision repair in prokaryotes and mammals

Biochem. J. (2010) Immediate Publication, doi:10.1042/BJ20101116

René Thierbach, Gunnar Drewes, Markus Fußer, Anja Voigt, Doreen Kuhlow, Urte Blume, Tim J Schulz, Carina Reiche, Hansruedi Glatt, Bernd Epe, Pablo Steinberg and Michael Ristow

Department of Human Nutrition, University of Jena, Jena 07743, Germany. 

 

Keywords:  DNA repair mechanisms,  iron-sulphur-clusters (ISCs),  frataxin, Friedreich’s Ataxia,  cancer, 8-oxoguanine glycosylase.

 

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Molecular details of the yeast frataxin-Isu1 interaction during mitochondrial Fe-S cluster assembly

Biochemistry, Just Accepted Manuscript, DOI: 10.1021/bi1008613,Publication Date (Web): September 3, 2010

 

Jeremy D. Cook , Kalyan C. Kondapalli , Swati Rawat , William C. Childs , Yogapriya Murugesan , Andrew Dancis , and Timothy Louis Stemmler 

 

Keywords: Iron Chaperone, Frataxin, Yfh1, Isu1, Nfs1, NMR, ITC, Iron-Sulfur Cluster Assembly.

 

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Protective effects of transduced PEP-1-Frataxin protein on oxidative stress-induced neuronal cell death

http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T06-50XV3JF-3&_user=10&_coverDate=09%2F03%2F2010&_rdoc=1&_fmt=high&_orig=browse&_origin=browse&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=2724eb2d4f21f0930a937b602939f104

Journal of the Neurological Sciences, In Press, Corrected Proof, Available online 3 September 2010,
Mi Jin Kim, Dae Won Kim, Ki-Yeon Yoo, Eun Jeong Sohn, Hoon Jae Jeong, Hye Won Kang, Min Jea Shin, Eun Hee Ahn, Jae Jin An, Soon Won Kwon, Young Nam Kim, Moo Ho Won, Sung-Woo Cho, Jinseu Park, Won Sik Eum and Soo Young Cho

Keywords:  Antioxidant; PEP-1-Frataxin; Protein transduction; Cell viability; Ischemia; ROS

New Research Demonstrates Safety Of Cord-blood-derived Stem Cell Treatments

Medical news Today, Article Date: 03 Sep 2010

In a new peer-reviewed article published by the Journal of Translational Medicine, scientists from Beike Biotechnology, China's leading stem cell research and regenerative medicine company, and Medistem, Inc., reported positive safety data in 114 patients who were treated by doctors at Nanshan Affiliated Hospital of Guangdong Medical College (Shenzhen Nanshan Hospital) in Shenzhen using Beike's proprietary cord blood stem cell transplantation protocol. ...read more...

Original source: Safety evaluation of allogeneic umbilical cord blood mononuclear cell therapy for degenerative conditions

Journal of Translational Medicine 2010, 8:75doi:10.1186/1479-5876-8-75

Wan-Zhang Yang1 email, Yun Zhang2 email, Fang Wu1 email, Wei-Ping Min3 email, Boris Minev4 email, Min Zhang1 email, Xiao-Ling Luo2 email, Famela Ramos5 email, Thomas E Ichim5 email, Neil H Riordan5* email and Xiang Hu2*


Background

The current paradigm for cord blood transplantation is that HLA matching and immune suppression are strictly required to prevent graft versus host disease (GVHD). Immunological arguments and historical examples have been made that the use of cord blood for non-hematopoietic activities such as growth factor production, stimulation of angiogenesis, and immune modulation may not require matching or immune suppression.

Methods

114 patients suffering from non-hematopoietic degenerative conditions were treated with non-matched, allogeneic cord blood. Doses of 1-3 × 107 cord blood mononuclear cells per treatment, with 4-5 treatments both intrathecal and intravenously were performed. Adverse events and hematological, immunological, and biochemical parameters were analyzed for safety evaluation.

Results

No serious adverse effects were reported. Hematological, immunological, and biochemical parameters did not deviate from normal ranges as a result of therapy.

Conclusion

The current hematology-based paradigm of need for matching and immune suppression needs to be revisited when cord blood is used for non-hematopoietic regenerative purposes in immune competent recipients.

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Protocol proposal for Friedreich ataxia molecular diagnosis using fluorescent and triplet repeat primed polymerase chain reaction

LINK: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B83WW-50X8GB2-1&_user=10&_coverDate=08%2F31%2F2010&_rdoc=1&_fmt=high&_orig=browse&_origin=browse&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=8b4ac06505c1ed6fa9282efeaeeed2fd

Translational Research. Article in Press.
doi:10.1016/j.trsl.2010.08.001

Mar Xunclàa, b, Laia Rodríguez-Revengaa, c, Irene Madrigala, c, Dolores Jiméneza, Montserrat Milàa, c, d and Cèlia Badenasa, c, d,

a Biochemistry and Molecular Genetics Service. Hospital Clínic, b Fundació Clínic per a la Recerca Biomèdica, c CIBER de Enfermedades Raras, d Institut d’Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain

Researchers Develop Hybrid Protein Tools For Gene Cutting And Editing

MedicalNews Today, Article Date: 31 Aug 2010

An Iowa State University team of researchers has developed a type of hybrid proteins that can make double-strand DNA breaks at specific sites in living cells, possibly leading to better gene replacement and gene editing therapies. Read more

[Distribution of frataxin in eye retina of normal mice and of transgenic R7E mice with retinal degeneration]

Zh Evol Biokhim Fiziol. 2010 Jul-Aug;46(4):347-9.

[Article in Russian]

Development of a potential therapy for Friedreich ataxia based on transduction of the frataxin protein in the mitochondria

Canadian Association for Familial Ataxias - Claude St-Jean Foundation
August 25, 2010,

CAFA IS LAUNCHING A MAJOR RESEARCH PROJECT

This research project’s goal is to develop a therapy for Friedreich ataxia by targeting the actual cause of the illness, the reduction of frataxin. The project will therefore aim to administer the frataxin protein intravenously. However, as this protein does not spontaneously penetrate cells, it will be encapsulated with peptides (fragments of other proteins), in nanoparticles. Alternatively, the frataxin protein itself will be modified by adding peptides which will allow the proteins to penetrate not only the interior of cells, but also the interior of the mitochondria.

Symposium participants optimistic about finding first treatment for Friedreich's ataxia

USF Health News, August 30, 2010 @ 4:58 pm

"With all the significant scientific advancements presented, at the end of the symposium it was the people with Friedreich’s ataxia who gave the research meaning and value"

Proteomic Analysis of Protein-Protein Interactions within the CSD Fe-S Cluster Biogenesis System

J. Proteome Res., Just Accepted Manuscript, Publication Date (Web): August 24, 2010

Heather May Bolstad , Danielle J Botelho and Matthew James Wood

Keygen: Fe-S cluster biogenesis, cysteine desulfurase CsdA, sulfur acceptor protein CsdE, E1-like protein CsdL. Fe-S cluster assembly (ErpA, glutaredoxin-3, glutaredoxin-4), sulfur trafficking (CsdL, YchN) proteins, two-pathway model.

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