Browsing by Subject "Lysosomes"
Now showing items 1-6 of 6
-
Adjunctive β2-agonist treatment reduces glycogen independently of receptor-mediated acid α-glucosidase uptake in the limb muscles of mice with Pompe disease.
(FASEB J, 2014-05)Enzyme or gene replacement therapy with acid α-glucosidase (GAA) has achieved only partial efficacy in Pompe disease. We evaluated the effect of adjunctive clenbuterol treatment on cation-independent mannose-6-phosphate ... -
Catch me if you can: the link between autophagy and viruses.
(PLoS pathogens, 2015-03-26) -
Lysosomal Disorders Drive Susceptibility to Tuberculosis by Compromising Macrophage Migration.
(Cell, 2016-03-24)A zebrafish genetic screen for determinants of susceptibility to Mycobacterium marinum identified a hypersusceptible mutant deficient in lysosomal cysteine cathepsins that manifests hallmarks of human lysosomal storage diseases. ... -
Preclinical characterization of DUOC-01, a cell therapy product derived from banked umbilical cord blood for use as an adjuvant to umbilical cord blood transplantation for treatment of inherited metabolic diseases.
(Cytotherapy, 2015-06)<h4>Background aims</h4>Cord blood (CB) transplantation slows neurodegeneration during certain inherited metabolic diseases. However, the number of donor cells in the brain of patients does not appear to be sufficient to ... -
Quality control autophagy: a joint effort of ubiquitin, protein deacetylase and actin cytoskeleton.
(Autophagy, 2010-05)Autophagy has been predominantly studied as a nonselective self-digestion process that recycles macromolecules and produces energy in response to starvation. However, autophagy independent of nutrient status has long been ... -
Stbd1 is highly elevated in skeletal muscle of Pompe disease mice but suppression of its expression does not affect lysosomal glycogen accumulation.
(Mol Genet Metab, 2013-07)Previous studies strongly suggest that starch binding domain containing protein 1 (Stbd1) plays an important role in intracellular glycogen trafficking into lysosomes. We report here that Stbd1 expression is markedly increased ...
