Poly glutamines

Beuckmann, C. T., Sinton, C. M., Williams, S. C. et al. (2004). Expression of a poly-glutamine-ataxin-3 transgene in orexin neurons induces narcolepsy-cataplexy in the rat. J. Neurosci 24, 4469-77. 

Waza M, Adachi H, Katsuno M, Minamiyama M, Sang C, Tanaka E Inukai A, Doyu M, Sobue G (2005) 17-AAG an Hsp90 inhibitor, ameliorates poly glutamine-mediated motor neuron degeneration. Nat Med 11 1088-1095. 

HD Huntingtin with poly-glutamin expansion Striatum, basal ganglia, cortex Intranuclear inclusions and cytoplasmic aggregates... 

Shoesmith Berke SJ, Flores Schmied FA, Brunt ER, Ellerby LM, Paulson HL (2004) Caspase-mediated proteolysis of the poly glutamine disease protein ataxin-3. J Neurochem 89 908-918 Smith R, Bacos K, Fedele V, Soulet D, Walz HA, Obermuller S, Lindqvist A, Bjdrkqvist M, Klein P, Onnerfjord P, Brandin P, Mulder H, Li JY (2009) Mutant huntingtin interacts with P-tubuUn and disrupts vesicular transport and insuhn secretion. Hum Mol Genet 18 3942-3954 Spindler M, Beal MF, Henchcliffe C (2009) Coenzyme QIO effects in neurodegenerative diseases. Neuropsychiatr Dis Treat 5 597-610... 

Increased huntingtin protein length reduces the severity of poly-glutamine-induced gene expression changes in mouse models of Huntington Disease. Hum Mol Genet 11 1939-1951. 

Steffan JS et al (2001) Histone deacetylase inhibitors arrest poly glutamine-dependent neurodegeneration in Drosophila. Nature 413(6857) 739-743... 

Friedman MJ et al (2007) Poly glutamine domain modulates the TBP-TFIIB interaction implications for its normal function and neurodegeneration. Nat Neurosci 10(12) 1519-1528... 

Helmlinger D et al (2006) Both normal and poly glutamine- expanded ataxin-7 are components of TFTC-type GCN5 histone acetyltransferase-containing complexes. Biochem Soc Symp 73 155-163... 

Terashima T et al (2002) SUMO-1 co-localized with mutant atrophin-1 with expanded poly glutamines accelerates intranuclear aggregation and cell death. NeuroReport 13(17) 2359-2364... 

Ryu H et al (2003) Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded poly glutamine repeats via an Spl-dependent pathway. Proc Natl Acad Sci USA 100(7) 4281-4286... 

Sun Y, Savanenin A, Reddy PH et al (2001) Poly glutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. J Biol Chem 276 24713-24718... 

Fig. 6 Excess energy from the water to the protein due to the presence of gin and asn for the prion (solid line) and for the poly-glutamine helix (dashed line), and excess energy transferred from the protein to the water in both proteins (thin line) (see text). Time is in ps.

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