How democracies prevail: democratic resilience as a two-stage process.

This article introduces a novel conceptualization of democratic resilience - a two-stage process where democracies avoid democratic declines altogether or avert democratic breakdown given that such autocratization is ongoing. Drawing on the Episodes of Regime Transformation (ERT) dataset, we find that democracies have had a high level of resilience to onset of autocratization since 1900. Nevertheless, democratic resilience has become substantially weaker since the end of the Cold War.

X-linked adrenomyeloneuropathy associated with 14 novel ALD-gene mutations: no correlation between type of mutation and age of onset.

Adrenomyeloneuropathy (AMN) represents a milder form of X-linked adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder. The disease is characterised by an abnormal accumulation of saturated, very long chain, fatty acids, because of altered peroxisomal beta-oxidation that concomitantly leads to demyelination in the central and peripheral nervous systems. ALD shows a highly variable phenotypic expression and extensive mutation analysis in ALD patients has failed to establish a genotype-phenotype correlation, even in the presence of the same ALD-gene defect.