SCA3: neurological features, pathogenesis and animal models
O Riess, U Rüb, A Pastore, P Bauer, L Schöls - The Cerebellum, 2008 - Springer
The most frequent subtype of autosomal dominant inherited spinocerebellar ataxias is
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …
Nicotinamide restores cognition in Alzheimer's disease transgenic mice via a mechanism involving sirtuin inhibition and selective reduction of Thr231-phosphotau
KN Green, JS Steffan, H Martinez-Coria… - Journal of …, 2008 - Soc Neuroscience
Memory loss is the signature feature of Alzheimer's disease, and therapies that prevent or
delay its onset are urgently needed. Effective preventive strategies likely offer the greatest …
delay its onset are urgently needed. Effective preventive strategies likely offer the greatest …
Modulation of sirtuins: new targets for antiageing
M Pallàs, E Verdaguer, M Tajes… - Recent Patents on …, 2008 - ingentaconnect.com
Aging is characterized by a progressive deterioration of physiological functions and
metabolic processes. Healthy aging remains one of the ideals of modern society. In aging …
metabolic processes. Healthy aging remains one of the ideals of modern society. In aging …
Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease
J Pallos, L Bodai, T Lukacsovich… - Human molecular …, 2008 - academic.oup.com
Huntington's disease (HD) is associated with transcriptional dysregulation, and multiple
studies with histone deacetylase (HDAC) inhibitors suggest that global approaches for …
studies with histone deacetylase (HDAC) inhibitors suggest that global approaches for …
[HTML][HTML] Polyglutamine Genes Interact to Modulate the Severity and Progression of Neurodegeneration in Drosophila
D Lessing, NM Bonini - PLoS biology, 2008 - journals.plos.org
The expansion of polyglutamine tracts in a variety of proteins causes devastating,
dominantly inherited neurodegenerative diseases, including six forms of spinal cerebellar …
dominantly inherited neurodegenerative diseases, including six forms of spinal cerebellar …
Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases
J Branco, I Al-Ramahi, L Ukani, AM Perez… - Human molecular …, 2008 - academic.oup.com
Abstract Spinocerebellar Ataxia type 1 (SCA1) and Huntington's disease (HD) are two
polyglutamine disorders caused by expansion of a CAG repeat within the coding regions of …
polyglutamine disorders caused by expansion of a CAG repeat within the coding regions of …
Hemolymph Amino Acid Analysis of Individual Drosophila Larvae
SC Piyankarage, H Augustin, Y Grosjean… - Analytical …, 2008 - ACS Publications
One of the most widely used transgenic animal models in biology is Drosophila
melanogaster, the fruit fly. Chemical information from this exceedingly small organism is …
melanogaster, the fruit fly. Chemical information from this exceedingly small organism is …
AKT-sensitive or insensitive pathways of toxicity in glial cells and neurons in Drosophila models of Huntington's disease
JC Lievens, M Iché, M Laval… - Human molecular …, 2008 - academic.oup.com
Huntington's disease (HD) is caused by an extended polyglutamine (polyQ) tract in the
Huntingtin protein. Neuronal and glial dysfunction precedes the neurodegeneration and …
Huntingtin protein. Neuronal and glial dysfunction precedes the neurodegeneration and …
Over‐expression of the catalytic core of mitochondrial DNA (mtDNA) polymerase in the nervous system of Drosophila melanogaster reduces median life span by …
F Martínez‐Azorín, M Calleja… - Journal of …, 2008 - Wiley Online Library
DNA polymerase γ (pol γ) is the sole DNA polymerase devoted to mitochondrial DNA
(mtDNA) replication. We have characterized the molecular and physiological effects of over …
(mtDNA) replication. We have characterized the molecular and physiological effects of over …
[HTML][HTML] A Functional Misexpression Screen Uncovers a Role for Enabled in Progressive Neurodegeneration
C Rezaval, J Berni, EA Gorostiza, S Werbajh… - PLoS …, 2008 - journals.plos.org
Drosophila is a well-established model to study the molecular basis of neurodegenerative
diseases. We carried out a misexpression screen to identify genes involved in …
diseases. We carried out a misexpression screen to identify genes involved in …