Autocracy-Sustaining Versus Democratic Federalism:Explaining the Divergent Trajectories of Territorial Politics in Russia and Western Europe

This article provides a comparative assessment of territorial politics in Russia and Western Europe. The consolidation or deepening of regional autonomy in Western Europe contrasts with the transformation of Russia from a segmented and highly centrifugal state into a centralized authoritarian state in the course of just two decades. The consolidation of territorial politics in Western Europe is linked to the presence of endogenous safeguards that are built into their territorial constitutional designs and most importantly to the dynamics that emanate from multi-level party competition in the context of a liberal and multi-level democracy. In contrast, in Russia, neither endogenous safeguards nor multi-level party democracy play an important role in explaining the dynamics of Russian federalism, but who controls key state resources instead. We argue that under Putin power dependencies between the Russian center and the regions are strongest where regional democracy is at its weakest, thus producing ‘autocracy-sustaining’ instead of a democratic federation. By studying the relationship between federalism and democracy in cases where both concepts are mutually reinforcing (as in Western Europe) with the critical case of Russia where they are not, we question the widely held view that democracy is a necessary pre-condition for federalism.Peer reviewe

Increased translation as a novel pathogenic mechanism in Huntington's disease

Huntington's disease is a neurodegenerative disorder caused by a CAG repeat expansion in exon 1 of the huntingtin gene. Striatal projection neurons are mainly affected, leading to motor symptoms, but molecular mechanisms involved in their vulnerability are not fully characterized. Here, we show that eIF4E binding protein (4E-BP), a protein that inhibits translation, is inactivated in Huntington's disease striatum by increased phosphorylation. Accordingly, we detected aberrant de novo protein synthesis. Proteomic characterization indicates that translation specifically affects sets of proteins as we observed upregulation of ribosomal and oxidative phosphorylation proteins and downregulation of proteins related to neuronal structure and function. Interestingly, treatment with the translation inhibitor 4EGI-1 prevented R6/1 mice motor deficits, although corticostriatal long-term depression was not markedly changed in behaving animals. At the molecular level, injection of 4EGI-1 normalized protein synthesis and ribosomal content in R6/1 mouse striatum. In conclusion, our results indicate that dysregulation of protein synthesis is involved in mutant huntingtin-induced striatal neuron dysfunction

PDGF-BB serum levels are decreased in adult onset Pompe patients

Adult onset Pompe disease is a genetic disorder characterized by slowly progressive skeletal and respiratory muscle weakness. Symptomatic patients are treated with enzymatic replacement therapy with human recombinant alfa glucosidase. Motor functional tests and spirometry are commonly used to follow patients up. However, a serological biomarker that correlates with the progression of the disease could improve follow-up. We studied serum concentrations of TGFβ, PDGF-BB, PDGF-AA and CTGF growth factors in 37 adult onset Pompe patients and 45 controls. Moreover, all patients performed several muscle function tests, conventional spirometry, and quantitative muscle MRI using 3-point Dixon. We observed a statistically significant change in the serum concentration of each growth factor in patients compared to controls. However, only PDGF-BB levels were able to differentiate between asymptomatic and symptomatic patients, suggesting its potential role in the follow-up of asymptomatic patients. Moreover, our results point to a dysregulation of muscle regeneration as an additional pathomechanism of Pompe disease