Fabio Russo

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We have previously demonstrated that gene therapy can rescue the phenotype and extend lifespan in the delta-sarcoglycan deficient cardiomyopathic hamster. In patients with similar genetic defects, steroids have been largely used to slow down disease progression. Aim of our study was to evaluate the combined effects of steroid treatment and gene therapy on(More)
Maroteaux-Lamy disease, also known as mucopolysaccharidosis (MPS) VI, is an MPS disorder caused by mutations in the ARSB gene encoding for the lysosomal enzyme arysulfatase B (ARSB). Deficient ARSB activity leads to lysosomal accumulation of dermatan sulfate in a wide range of tissues and organs. There are various animal models of MPS VI that have been well(More)
Location and stability of a recombinant prion protein (recPrP) and its interaction with humic-like complexes were investigated by low-temperature ashing (LTA), thermal gravimetric (TG), and scanning electron microscopy (SEM) analyses. Humic-like complexes were obtained by abiotic polymerization of catechol, one of the possible precursors of soil humic(More)
Information-theory provides, among others, conceptual methods to quantify the amount of information contained in single random variables and methods to quantify the amount of information contained and shared among two or more variables. Although these concepts have been successfully applied in hydrology and other fields, the evaluation of these quantities(More)
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