... and neurodegeneration is outlined. Finally, outstanding questions and hypotheses in this field are proposed. Chromatin state models suggest that polycomb proteins are responsible for neuronal subtype specification rather than broad repression of non-neuronal transcriptional programmes. In support of this idea...

... (TSEN) complex display overlapping phenotypes in both vertebrates and invertebrates. RNA processing Animal models of human disease Neurodegeneration tRNA splicing National Institutes of Health http://dx.doi.org/10.13039/100000002 R35-GM136435 DGE-1650116 National Science...

... Neurodegeneration Neuromuscular junction Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disease characterized by the progressive loss of motor neurons ( Boillee et al., 2006 ). Post-diagnosis survival times average 3–5 years and currently there is no treatment available...

... metalloproteases (MMPs) production, which in turn promote further TMs infiltration, GB progression and neurodegeneration. Thus, cellular and molecular signals other than primary mutations emerge as central players of GB. Using a Drosophila model of GB, we describe the temporal organisation of the main cellular...

... are consistent with the hypothesis that induction of autophagy downstream of AMPK-mediated tau phosphorylation is beneficial in reducing tau-dependent neurodegeneration. The exact role of AMPK in neurodegeneration is still highly controversial. It has been reported that a beneficial outcome of AMPK...

.... Using axonal transport of the morphogen Hedgehog (Hh), which is integral to Dmel eye-brain development and implicated in stem cell maintenance and neoplastic disease, we demonstrate the ability to comprehensively quantify and characterize its trafficking in various neuron types and a neurodegeneration...

... of Neurological Disorders and Stroke (NINDS) (R01 NS33202 to C.M.G. and R01 NS086778 to S.V.T.). References Bilen , J. and Bonini , N. M. ( 2007 ). Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila . PLoS Genet. 3 , e177 . 10.1371/journal.pgen.0030177...

... A. , Strand A. , Torcassi C. , Savage J. , Hurlburt A. ( 2007 ). Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet. 3 , e82 . 10.1371/journal.pgen.0030082 Kambris Z. , Brun S. , Jang I. H. , Nam H. J. , Romeo Y...

... Neurodegeneration Genetic modifiers Wolfram syndrome Copy number variants Lysosomal storage disease Gene network Wolfram syndrome (WFS) is an autosomal recessive neurodegenerative disease that affects 1 in 770,000 people in the United Kingdom ( Barrett et al., 1995 ). Affected individuals present...

... locomotion behavior, nuclear abnormalities, neurodegeneration and upregulation of the heat-shock-mediated stress response. Similar, albeit milder, phenotypes are associated with the overexpression of the wild-type protein. These data show that overexpressing the wild-type DVAP is sufficient to induce...

... of CLN6 brains. These data are consistent with a link between CLN6 expression and biometal homeostasis in CLN6 disease, and provide further support for altered cation transporter regulation as a key factor in neurodegeneration. The metal content in 5 regions of the mouse CNS and 3 peripheral mouse...