Coenzyme Q10 and Neurological Diseases

Pharmaceuticals 2009, 2, 134-149; doi:10.3390/ph203134

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Michelangelo Mancuso *, Daniele Orsucci, Valeria Calsolaro, Anna Choub and Gabriele Siciliano
Department of Neuroscience, Neurological Clinic, University of Pisa, Tuscany, Italy

Abstract: Coenzyme Q10 (CoQ10, or ubiquinone) is a small electron carrier of the mitochondrial respiratory chain with antioxidant properties. CoQ10 supplementation has been widely used for mitochondrial disorders. The rationale for using CoQ10 is very powerful when this compound is primary decreased because of defective synthesis. Primary CoQ10 deficiency is a treatable condition, so heightened “clinical awareness” about this diagnosis is essential. CoQ10 and its analogue, idebenone, have also been widely used in the treatment of other neurodegenerative disorders. These compounds could potentially play a therapeutic role in Parkinson’s disease, Huntington’s disease, amyotrophic lateral sclerosis, Friedreich’s ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of CoQ10, as well as the rationale and the role in clinical practice of CoQ10 supplementation in different neurological diseases, from primary CoQ10 deficiency to neurodegenerative disorders.
Keywords: coenzyme Q10;  idebenone;  mitochondria; mitochondrial diseases; neurodegeneration

Can rehabilitation help ataxia?

Neurology, Vol. 73, Issue 22, 1818-1819, December 1, 2009 , EDITORIALS
Susanne M. Morton and Amy J. Bastian

Intensive coordinative training improves motor performance in degenerative cerebellar disease

NEUROLOGY 2009;73:1823-1830

W. Ilg, PhD, M. Synofzik, MD, D. Brötz, S. Burkard, M. A. Giese, PhD and L. Schöls, MD


From the Departments of Cognitive Neurology (W.I., M.A.G.) and Neurodegeneration (M.S., L.S.), Hertie Institute for Clinical Brain Research and Center of Neurology, Tübingen; Institute of Medical Psychology and Behavioral Neurobiology (D.B.), MEG Center, University Tübingen; and Therapy Centre (S.B.), Center of Neurology, University Clinic Tübingen, Germany.

Keywords: cerebellum, motor control, motor learning, physiotherapeutic training, cerebellar degeneration,  clinical ataxia rating scales, individual goal attainment scores, quantitative movement analysis, gait like velocity, lateral sway, intralimb coordination, standard of care, Berg balance score, goal attainment score, ICARS, international cooperative ataxia rating scale, SARA, scale for the assessment and rating of ataxia.

HDAC6 is a target for protection and regeneration following injury in the nervous system.

Proc Natl Acad Sci U S A. 2009 Nov 17;106(46):19599-604. Epub 2009 Nov 2.

Rivieccio MA, Brochier C, Willis DE, Walker BA, D'Annibale MA, McLaughlin K, Siddiq A, Kozikowski AP, Jaffrey SR, Twiss JL, Ratan RR, Langley B.
Burke Medical Research Institute, 785 Mamaroneck Avenue, White Plains, NY 10605, USA.

Keywords:Central nervous system (CNS) trauma,, neuronal degeneration, axonal degeneration, toxicity, anticancer properties, neuronal protection against oxidative stress,  promotes neurite growth,  HDAC6.


 

Metal chelators coupled with nanoparticles as potential therapeutic agents for Alzheimer's disease.

J Nanoneurosci. 2009 Jun 1;1(1):42-55.

Liu G, Men P, Perry G, Smith MA.

Department of Radiology, University of Utah, Salt Lake City, UT 84108, USA.

Keywords: Alzheimer's disease (AD), blood-brain barrier,  nanoparticle-mediated drug delivery,  chelation agents,  iron-associated neurodegenerative diseases, Friedreich's ataxia, Parkinson's disease, Huntington's disease,  Hallervorden-Spatz Syndrome, nanoparticle technology, very early stages of development.

Clinical aspects of coenzyme Q(10): An update.

Nutrition. 2009 Nov 20.

Littarru GP, Tiano L.
Department of Biochemistry, Biology and Genetics, Polytechnic University of the Marche, Ancona, Italy.

Keywords:  Q(10) (CoQ(10)),  in mitochondrial bioenergetics,  antioxidant properties, clinical applications, cardiovascular disease, physical exercise, mitochondrial myopathies, Parkinson's and Huntington's diseases,  Friedreich's ataxia,  preeclampsia in pregnancy. headache symptoms, pediatric and adolescent populations.

A theoretical analysis on characteristics of protein structures induced by cold denaturation

J. Chem. Phys. 131, 205102 (2009); doi:10.1063/1.3265985

Published 24 November 2009

Hiraku Oshima, Takashi Yoshidome, Ken-ichi Amano, and Masahiro Kinoshita

Institute of Advanced Energy, Kyoto University, Uji, Kyoto 611-0011, Japan

Keywords:  frataxin, cold denaturation,  morphometric approach, entropy change,  unfolded structures.

A Diet Enriched in Polyphenols and Polyunsaturated Fatty Acids, LMN Diet, Induces Neurogenesis in the Subventricular Zone and Hippocampus of Adult Mouse Brain

Journal of Alzheimer's Disease, IOS Press, DOI 10.3233/JAD-2009-1188
Authors


Tony Valente1, Juan Hidalgo2, Irene Bolea1, Bartolomé Ramirez3, Neus Anglés3, Jordi Reguant3, José Ramón Morelló3, Cristina Gutiérrez1, Mercè Boada4, Mercedes Unzeta1

1Departament de Bioquimica i Biologia Molecular, Institut de Neurociències, Facultat de Medicina, Torre M2, Universitat Autònoma de Barcelona, Bellaterra, Barcelona, Spain
2Institut de Neurociències, and Departamento de Biología Celular, Fisiologia, e Inmunología, Facultat de Biociències, Universitat Autònoma de Barcelona, Bellaterra, Barcelona, Spain
3La Morella Nuts SA, Reus, Tarragona, Spain
Fundació ACE, Institut Català de Neurociències Aplicades, Barcelona, Spain

Keywords: 129S1/SvImJ mice, adult neurogenesis, diet, hippocampus, olfactory bulb, polyphenols, polyunsaturated fatty acids, several neurodegenerative pathologies, potent antioxidant effect, neurodegenerative diseases.

Full text:  http://iospress.metapress.com/content/91261l2n31q4q797/fulltext.pdf

Polyphenols and Polyunsaturated Fatty Acids Boost the Birth of New Neurons, Study Finds

ScienceDaily (Nov. 24, 2009) — Universitat Autònoma de Barcelona (UAB) researchers have confirmed that a diet rich in polyphenols and polyunsaturated fatty acids, patented as an LMN diet, helps boost the production of the brain's stem cells -neurogenesis- and strengthens their differentiation in different types of neuron cells. read more....

Histone modifications are specifically relocated during gene activation and nuclear differentiation

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Katharina S Heyse , Susanne E Weber and Hans Joachim Lipps

BMC Genomics 2009, 10:554doi:10.1186/1471-2164-10-554
Published: 24 November 2009

Abstract (provisional)

Background
Post-translational histone modifications (PTMs) and their specific distribution on genes play a crucial role in the control of gene expression, but the regulation of their dynamics upon gene activation and differentiation is still poorly understood. Here, we exploit the unique genome organization of ciliates to analyse PTM dynamics during gene activation in the differentiated cell and during nuclear differentiation. In the macronucleus of these cells the DNA is organized into nanochromosomes which represent independent functional units. Therefore ciliated protozoa represent a simplistic model system to analyse the relevance of histone modifications and their localization for gene expression and differentiation.

Results
We analysed the distribution of three PTMs on six individual nanochromosomes, two of which are silenced in the vegetative cell and only activated during sexual reproduction. We show that a specific relocation of these PTMs correlates with gene activation. Moreover, macronuclear-destined sequences in the differentiating macronucleus display a distribution of PTMs which differs significantly from the PTM patterns of actively transcribed genes.

Conclusions
We show for the first time that a relocation of specific histone modifications takes place during activation of genes. In addition, we demonstrate that genes in a differentiating nucleus are characterised by a specific distribution and composition of PTMs. This allows us to propose a mechanistic model about the relevance of PTMs for gene activation, gene silencing and nuclear differentiation. Results described here will be relevant for eukaryotic cells in general.

The complete article is available as a provisional PDF