1,615 publications from this institution
Rett Syndrome is a neurodevelopmental autism spectrum disorder caused by mutations in the gene coding for methyl CpG-binding protein (MeCP2). The disease is characterized by abnormal motor, respiratory, cognitive impairment, and autistic-like behaviors. No effective treatment of the disorder is available. Mecp2 knockout mice have a range of physiological and neurological abnormalities that resemble the human syndrome and can be used as a model to interrogate new therapies. Herein, we show that the combined administration of Levodopa and a Dopa-decarboxylase inhibitor in RTT mouse models is well tolerated, diminishes RTT-associated symptoms, and increases life span. The amelioration of RTT symptomatology is particularly significant in those features controlled by the dopaminergic pathway in the nigrostratium, such as mobility, tremor, and breathing. Most important, the improvement of the RTT phenotype upon use of the combined treatment is reflected at the cellular level by the development of neuronal dendritic growth. However, much work is required to extend the duration of the benefit of the described preclinical treatment.
Ascertaining the cause/s of differences in performance between units of the public administration is at least as important as quantifying the very level of individual performance. We identify two causes: inefficiency and con- gestion. Inefficiency might be due to the fact that inputs are of relatively low quality (e.g, temporary workers) and/or, for a given quality, to the fact that inputs do not have incentives to exert their maximum level of effort. Congestion arises when the number of new cases is above the number that can be solved when full efficiency is achieved. This decomposition is ap- plied to the universe of Catalonian first instance courts for the period 2005-13 applying a fixed-effect panel stochastic frontier model (Wang and Ho, 2010). In this particular case, we conclude that poor performance is not due to inefficiency on the courts' side, but to an increase in the litigation demand, that is, to congestion, while inefficiency tends to decrease along time and is correlated with the presence of temporary judges.
