The Lancet, Volume 376, Issue 9757, Pages 1975 - 1976, 11 December 2010, doi:10.1016/S0140-6736(10)62247-7
Nellie Bristol
Commission Chair Amy Gutmann, President of the University of Pennsylvania stated. “We have an ethical obligation to protect the health and well-being of all research participants.”
Sunday, December 12, 2010
Thursday, December 9, 2010
Electrophysiology of Respiratory Chain Complexes and the ADP-ATP Exchanger in Native Mitochondrial Membranes.
Biochemistry. 2010 Dec 7;49(48):10308-18. Epub 2010 Nov 11.
Watzke N, Diekert K, Obrdlik P.
IonGate Biosciences GmbH, Industriepark Hoechst, D528, 65926 Frankfurt am Main, Germany.
Keywords: mitochondrial membranes, proton-pumping respiratory chain complexes, mitochondrial secondary active solute transport proteins, solid-supported membrane (SSM) technology, respiratory chain complexes CI, CII, CIII, and CIV, the F(O)F(1)-ATPase/synthase (CV), the adenine nucleotide translocase (ANT), oxidative phosphorylation (OXPHOS), uncoupler carbonyl cyanide m-chlorophenylhydrazone (CCCP), IC(50), Coenzyme Q (CoQ), decylubiquinone (DBQ), idebenone (Ide).
Watzke N, Diekert K, Obrdlik P.
IonGate Biosciences GmbH, Industriepark Hoechst, D528, 65926 Frankfurt am Main, Germany.
Keywords: mitochondrial membranes, proton-pumping respiratory chain complexes, mitochondrial secondary active solute transport proteins, solid-supported membrane (SSM) technology, respiratory chain complexes CI, CII, CIII, and CIV, the F(O)F(1)-ATPase/synthase (CV), the adenine nucleotide translocase (ANT), oxidative phosphorylation (OXPHOS), uncoupler carbonyl cyanide m-chlorophenylhydrazone (CCCP), IC(50), Coenzyme Q (CoQ), decylubiquinone (DBQ), idebenone (Ide).
Wednesday, December 8, 2010
Axial diffusivity is increased in the degenerating superior cerebellar peduncles of Friedreich's ataxia.
Neuroradiology. 2010 Dec 3. [Epub ahead of print]
Della Nave R, Ginestroni A, Diciotti S, Salvatore E, Soricelli A, Mascalchi M., S. Giuseppe Hospital, Radiodiagnostic Section, Empoli, Italy.
Keywords: Decreased fractional anisotropy (FA), diffusion tensor MR imaging (DTI), white matter (WM), radial diffusivity, axial diffusivity, Friedreich's ataxia (FRDA), selective neuronal loss, dentate nuclei, cerebellar peduncles (SCPs), TBSS analysis.
Della Nave R, Ginestroni A, Diciotti S, Salvatore E, Soricelli A, Mascalchi M., S. Giuseppe Hospital, Radiodiagnostic Section, Empoli, Italy.
Keywords: Decreased fractional anisotropy (FA), diffusion tensor MR imaging (DTI), white matter (WM), radial diffusivity, axial diffusivity, Friedreich's ataxia (FRDA), selective neuronal loss, dentate nuclei, cerebellar peduncles (SCPs), TBSS analysis.
Tuesday, December 7, 2010
Large-scale in silico modeling of metabolic interactions between cell types in the human brain
Nature Biotechnology, Year published:(2010), DOI: doi:10.1038/nbt.1711
Nathan E Lewis, Gunnar Schramm, Aarash Bordbar, Jan Schellenberger, Michael P Andersen, Jeffrey K Cheng, Nilam Patel, Alex Yee, Randall A Lewis, Roland Eils, Rainer König, Bernhard Ø Palsson.
Keywords: Metabolic interactions, gene expression data, proteomics data, literature-based manual curation, model human metabolism, metabolites, interstitial fluid, models of brain energy metabolism, astrocytes, Alzheimer's disease, regions of the brain.
Simplest explanation: Metabolism Models May Explain Why Alzheimer's Disease Kills Some Neuron Types First (ScienceDaily (Dec. 6, 2010)
Nathan E Lewis, Gunnar Schramm, Aarash Bordbar, Jan Schellenberger, Michael P Andersen, Jeffrey K Cheng, Nilam Patel, Alex Yee, Randall A Lewis, Roland Eils, Rainer König, Bernhard Ø Palsson.
Keywords: Metabolic interactions, gene expression data, proteomics data, literature-based manual curation, model human metabolism, metabolites, interstitial fluid, models of brain energy metabolism, astrocytes, Alzheimer's disease, regions of the brain.
Simplest explanation: Metabolism Models May Explain Why Alzheimer's Disease Kills Some Neuron Types First (ScienceDaily (Dec. 6, 2010)
Monday, December 6, 2010
Taking the lottery out of gene therapy
NEW SCIENTIST HEALTH ,Magazine issue 2789
GENE therapy should become a more exact science thanks to the discovery that it is possible to predict where a transferred gene is likely to be inserted into the recipient's DNA.
"Peter Cherepanov at Imperial College London, who was not part of the team, says now that the probability of an undesirable insertion can be estimated, it will become easier to balance the chance of success with the risk of side effects."
ORIGINAL PAPER: Deciphering the Code for Retroviral Integration Target Site Selection
FULL TEXT PDF
GENE therapy should become a more exact science thanks to the discovery that it is possible to predict where a transferred gene is likely to be inserted into the recipient's DNA.
"Peter Cherepanov at Imperial College London, who was not part of the team, says now that the probability of an undesirable insertion can be estimated, it will become easier to balance the chance of success with the risk of side effects."
ORIGINAL PAPER: Deciphering the Code for Retroviral Integration Target Site Selection
FULL TEXT PDF
Catalysis and Mechanistic Insights into Sirtuin Activation.
Chembiochem. 2010 Nov 9. [Epub ahead of print]
Dittenhafer-Reed KE, Feldman JL, Denu JM.
Department of Biomolecular Chemistry, School of Medicine and Public Health, University of Wisconsin-Madison.
Keywords: SIRT1, resveratrol, SRT1720, mechanism by which they activate remains poorly defined, type II diabetes, neurodegeneration, diseases associated with aging.
Repeat expansion affects both transcription initiation and elongation in friedreich ataxia cells
J Biol Chem. 2010 Dec 2. [Epub ahead of print]
Kumari D, Biacsi RE, Usdin K., NIH, United States.
Keywords: Expansion of a GAA·TTC-repeat, first intron, frataxin (FXN) gene, mRNA deficit, Friedreich ataxia (FRDA), DNA methylation, histone modifications, chromatin immuno-precipitation, chromatin, RNA polymerase II, histone H3 trimethylated on lysine 4, trimethylated H3K36.
Kumari D, Biacsi RE, Usdin K., NIH, United States.
Keywords: Expansion of a GAA·TTC-repeat, first intron, frataxin (FXN) gene, mRNA deficit, Friedreich ataxia (FRDA), DNA methylation, histone modifications, chromatin immuno-precipitation, chromatin, RNA polymerase II, histone H3 trimethylated on lysine 4, trimethylated H3K36.
Sunday, December 5, 2010
Accelerating orphan drug development
Nature Reviews Drug Discovery 9, 901-902 (December 2010) | doi:10.1038/nrd3340
Timothy R. Coté, Kui Xu & Anne R. Pariser
"Given the limited resources available for rare disease R&D, it is imperative that all knowledge gained is used to maximum benefit at each phase.
The FDA is committed to accelerating orphan drug development through a regulatory system built on integrity, consistency and transparency; a system that has delivered benefits to people who desperately need them and promises to deliver much more."
Timothy R. Coté, Kui Xu & Anne R. Pariser
"Given the limited resources available for rare disease R&D, it is imperative that all knowledge gained is used to maximum benefit at each phase.
The FDA is committed to accelerating orphan drug development through a regulatory system built on integrity, consistency and transparency; a system that has delivered benefits to people who desperately need them and promises to deliver much more."
Saturday, December 4, 2010
The Neuropathology of Late-Onset Friedreich's Ataxia.
Cerebellum. 2010 Dec 4. [Epub ahead of print]
Koeppen AH, Morral JA, McComb RD, Feustel PJ.
Research Service (151), VA Medical Center, 113 Holland Ave, Albany, NY, 12208, USA.
Keywords: Friedreich's ataxia (FRDA), very young persons, routine laboratory test, cardiomyopathy, late-onset, neurological disability, dorsal root ganglia, atrophy of the dentate nucleus, Frataxin levels.
Koeppen AH, Morral JA, McComb RD, Feustel PJ.
Research Service (151), VA Medical Center, 113 Holland Ave, Albany, NY, 12208, USA.
Keywords: Friedreich's ataxia (FRDA), very young persons, routine laboratory test, cardiomyopathy, late-onset, neurological disability, dorsal root ganglia, atrophy of the dentate nucleus, Frataxin levels.
Wednesday, December 1, 2010
Mitochondrial Fe-S cluster biogenesis, frataxin and the modulation of susceptibility to drug-induced cardiomyopathy
Aging (Albany NY). 2010 Nov 27.
Michael N. Sack
NHLBI Center for Molecular Medicine, National Institutes of Health, Bethesda, MD 20892, USA
Commentary on: Schulz et al. Activation of mitochondrial energy metabolism protects against cardiac failure. Aging 2010; 2: this issue
OPEN ACCESS
An intriguing new finding, by Shultz et al is published in AGING regarding the induction of frataxin. Mutations in frataxin result in the development of Friedreich's Ataxia, an inherited neurodegenerative disease associated with the development of severe cardiomyopathy. Frataxin, itself is involved in mitochondrial iron-sulphur cluster biogenesis which functions, in part, to incorporate appropriate amounts of iron into mitochondrial proteins including aconitase and succinate dehydrogenase [13]. Whether frataxin functions as an iron-chaperone protein or plays a regulatory role in controlling iron and sulphur flux within mitochondria is not yet completely characterized. Nevertheless, the study by Shultz and colleagues [14] shows that increased cardiac frataxin enhances tricarboxylic acid cycle function resulting in increased cardiac ATP, NADH, NADPH and reduced glutathione levels. This array of features is consistent with an enhanced bioenergetic capacity and increased antioxidant defenses. The authors go on to demonstrate that overexpression of frataxin is cardioprotective against doxorubicin-induced cardiomyopathy. This intriguing study shows that the modulation of the mitochondria at the fundamental level of integrating cofactors required for protein functional integrity have beneficial effects in disease processes that are exacerbated by mitochondrial dysfunction. This study further highlights the complexity of mitochondrial function and adds a new level of regulation operational in the pathophysiology of heart failure that may be amenable to therapeutic modulation.
FULL TEXT PDF
Michael N. Sack
NHLBI Center for Molecular Medicine, National Institutes of Health, Bethesda, MD 20892, USA
Commentary on: Schulz et al. Activation of mitochondrial energy metabolism protects against cardiac failure. Aging 2010; 2: this issue
OPEN ACCESS
An intriguing new finding, by Shultz et al is published in AGING regarding the induction of frataxin. Mutations in frataxin result in the development of Friedreich's Ataxia, an inherited neurodegenerative disease associated with the development of severe cardiomyopathy. Frataxin, itself is involved in mitochondrial iron-sulphur cluster biogenesis which functions, in part, to incorporate appropriate amounts of iron into mitochondrial proteins including aconitase and succinate dehydrogenase [13]. Whether frataxin functions as an iron-chaperone protein or plays a regulatory role in controlling iron and sulphur flux within mitochondria is not yet completely characterized. Nevertheless, the study by Shultz and colleagues [14] shows that increased cardiac frataxin enhances tricarboxylic acid cycle function resulting in increased cardiac ATP, NADH, NADPH and reduced glutathione levels. This array of features is consistent with an enhanced bioenergetic capacity and increased antioxidant defenses. The authors go on to demonstrate that overexpression of frataxin is cardioprotective against doxorubicin-induced cardiomyopathy. This intriguing study shows that the modulation of the mitochondria at the fundamental level of integrating cofactors required for protein functional integrity have beneficial effects in disease processes that are exacerbated by mitochondrial dysfunction. This study further highlights the complexity of mitochondrial function and adds a new level of regulation operational in the pathophysiology of heart failure that may be amenable to therapeutic modulation.
FULL TEXT PDF
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