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 …

Choosing and using Drosophila models to characterize modifiers of Huntington's disease

EW Green, F Giorgini - Biochemical Society Transactions, 2012 - portlandpress.com
HD (Huntington's disease) is a fatal inherited gain-of-function disorder caused by a polyQ
(polyglutamine) expansion in the htt (huntingtin protein). Expression of mutant htt in model …

[PDF][PDF] Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice

X Gu, C Li, W Wei, V Lo, S Gong, SH Li, T Iwasato… - Neuron, 2005 - cell.com
Expanded polyglutamine (polyQ) proteins in Huntington's disease (HD) as well as other
polyQ disorders are known to elicit a variety of intracellular toxicities, but it remains unclear …

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 …

Interacting proteins as genetic modifiers of Huntington disease

XJ Li, M Friedman, S Li - Trends in Genetics, 2007 - cell.com
Huntington disease is caused by polyglutamine expansion in huntingtin, a 350kD protein
that is ubiquitously expressed and widely distributed at the subcellular level. Recently …

Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease

CE Wang, S Tydlacka, AL Orr, SH Yang… - Human molecular …, 2008 - academic.oup.com
A number of mouse models expressing mutant huntingtin (htt) with an expanded
polyglutamine (polyQ) domain are useful for studying the pathogenesis of Huntington's …

Modeling Huntington disease in Drosophila Insights into axonal transport defects and modifiers of toxicity

M Krench, JT Littleton - Fly, 2013 - Taylor & Francis
Huntington disease (HD) is an inherited neurodegenerative disorder caused by a
polyglutamine (polyQ) expansion in the huntingtin (Htt) gene. Despite years of research …

Protective role of Engrailed in a Drosophila model of Huntington's disease

B Mugat, ML Parmentier, N Bonneaud… - Human molecular …, 2008 - academic.oup.com
Huntington's disease (HD) is caused by the expansion of the polyglutamine (polyQ) tract in
the human Huntingtin (hHtt) protein (polyQ-hHtt). Although this mutation behaves …

13 Mouse models of Huntington's disease

GP Bates, KPSJ Murphy - Huntington's disease, 2002 - books.google.com
The isolation of the Huntington's disease (HD) gene in 1993 (Huntington's Disease
Collaborative Research Group 1993) made it possible to generate in vivo genetic models of …

Serine residues 13 and 16 are key modulators of mutant huntingtin induced toxicity in Drosophila

M Chatterjee, JS Steffan, T Lukacsovich, JL Marsh… - Experimental …, 2021 - Elsevier
Poly-glutamine expansion near the N-terminus of the huntingtin protein (HTT) is the prime
determinant of Huntington's disease (HD) pathology; however, post-translational …