Lysosomal storage disorders (LSDs) are a group of ~50 genetic diseases of lysosomal function resulting in an intra-lysosomal accumulation of undegraded material with a combined incidence of 1:5.000 live births (Fuller et al. 2006). The clinical phenotype of LSDs varies widely from skeletal and visceral manifestation to severe CNS involvement depending on the defective protein and storage material involved.

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The lungs are the vital organs in charge of delivering oxygen to the cells. At the same time they also protect the organism from many threats contained in the air such as pollutants and pathogens. The mechanism by which these functions are achieved is extraordinarily complex to be explained in short,

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