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This paper investigates an innovative open thermochemical system dedicated to high density and long term (seasonal) storage purposes. It involves a hydrate/water reactive pair and operates with moist air. This work focuses on the design of and experimentation with a large scale prototype using SrBr 2 /H 2 O as a reactive pair (400 kg of hydrated salt, 105 kWh of storage capacity and a reactor energy density of 203 kWh/m 3). Promising conclusions have been obtained regarding the feasibility and performance of such a storage process. Hydration specific powers from 0.75 to 2 W/kg have been reached for a bed salt energy density of 388 kWh/m 3. Moreover, two important parameters that control the storage system have been identified and investigated: the equilibrium drop and the mass flow rate of moist air. Both have a strong influence on the reaction kinetics and therefore on the reactor's thermal power.

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Spinal muscular atrophy (SMA) is an autosomal-recessive pediatric neurodegenerative disease characterized by selective loss of spinal motor neurons. It is caused by mutation in the survival of motor neuron 1, SMN1, gene and leads to loss of function of the full-length SMN protein. microRNAs (miRNAs) are small RNAs that are involved in post-transcriptional regulation of gene expression. Prior studies have implicated miRNAs in the pathogenesis of motor neuron disease. We hypothesized that motor neuron-specific miRNA expression changes are involved in their selective vulnerability in SMA. Therefore, we sought to determine the effect of SMN loss on miRNAs and their target mRNAs in spinal motor neurons. We used microarray and RNAseq to profile both miRNA and mRNA expression in primary spinal motor neuron cultures after acute SMN knockdown. By integrating the miRNA:mRNA profiles, a number of dysregulated miRNAs were identified with enrichment in differentially expressed putative mRNA targets. miR-431 expression was highly increased, and a number of its putative mRNA targets were significantly downregulated in motor neurons after SMN loss. Further, we found that miR-431 regulates motor neuron neurite length by targeting several molecules previously identified to play a role in motor neuron axon outgrowth, including chondrolectin. Together, our findings indicate that cell-type-specific dysregulation of miR-431 plays a role in the SMA motor neuron phenotype.