Both neuroprotective and neurotoxic roles have previously been described for histone
deacetylase-1 (HDAC1). Here we report that HDAC1 expression is elevated in vulnerable …

Background An array of experimental models have been developed in the small model
organisms C. elegans, S. cerevisiae and D. melanogaster for the study of various …

Proteins with long, pathogenic polyglutamine (polyQ) sequences have an enhanced
propensity to spontaneously misfold and self-assemble into insoluble protein aggregates …

The fruit fly (Drosophila melanogaster) is an extensively used and powerful, genetic model
organism. However, chemical studies using individual flies have been limited by the …

Severe reduction in Survival Motor Neuron 1 (SMN1) protein in humans causes Spinal
Muscular Atrophy (SMA), a debilitating childhood disease that leads to progressive …

Ataxia-telangiectasia (AT) is a multifaceted disorder caused by mutations in the ataxia-
telangiectasia mutated (ATM) gene. ATM encodes a serine-threonine protein kinase that …

1.1 Autosomal dominant cerebellar ataxias type I 9 1.1. 1 Triplet repeat disorders 9 1.1. 2
Pathomechanism of polyglutamine disorders 10 1.1. 3 Spinocerebellar ataxia type 2 12 1.2 …

Amyloid-beta (Aβ) plaques are a major neuropathological feature of Alzheimer's disease
(AD). These plaques are primarily composed of aggregates of Aβ peptides generated via the …