Inflammation causes significant morbidity and mortality, necessitating effective in vivo imaging of inflammation. Prior approaches often rely on combination of optical agents with entities specific for proteinaceous biomarkers overexpressed in inflammatory tissues. We herein report a fundamentally new approach to...
Its sites of glycosylation have been reviewed in the model system of Chinese hamster ovary and human embryonic kidney 293 cells (35) but not in platelets. Therefore, results gained in the one system may not be readily transferable to the other. Download: Download full-size image Fig. 4. In...
P. et al. Autophagy in lysosomal storage disorders. Autophagy 8, 719–730 (2012). Article CAS PubMed PubMed Central Google Scholar Takikita, S. et al. The values and limits of an in vitro model of Pompe disease: the best laid schemes o’mice an’men …. Autophagy 5, 729–731 (...
We report a novel loss-of-function mutation in the TLDc domain of the humanOXR1gene, resulting in early-onset epilepsy, developmental delay, cognitive disabilities, and cerebellar atrophy. Patient lymphoblasts show impaired cell survival, proliferation, and hypersensitivity to oxidative stress. These ...
uP, number of unique peptide sequences identified for each protein. Subcellular location: C, cytoplasmic; CAV, caveolae; CSK, cytoskeletal; ER, endoplasmic reticulum; EX, extracellular; G, Golgi complex; L, lysosomal; Me, plasma membrane; M, mitochondrial; MIM, mitochondrial inner membrane; MIMS...
We find that endo-lysosomal pH in fact strongly accelerates AβO formation, whereas amyloid fibril formation is delayed, suggesting that AβO formation is the dominant aggregation process in endosomes/lysosomes. We furthermore show that dimAβ is a disease-relevant model construct for pathogenic A...
The findings are relevant to diseases with reduced RNA editing and increased circRNA levels and provide insights into the human-specific regulation of circRNA formation.Similar content being viewed by others Striated muscle-specific base editing enables correction of mutations causing dilated cardiomyopathy ...
(PH062) Lysosomal destabilization as a screening tool in large-scale biomonitoring.Wade, TL
Age-related macular degeneration (AMD) is the leading cause of blindness among the elderly. Dry AMD has unclear etiology and no treatment. Lipid-rich drusen are the hallmark of dry AMD. An AMD mouse model and insights into drusenogenesis are keys to better understanding of this disease. Chlor...
Retromer stabilization results in neuroprotection in a model of Amyotrophic Lateral Sclerosis Luca Muzio, Riccardo Sirtori, Davide Gornati, Simona Eleuteri, Andrea Fossaghi, Diego Brancaccio, Leonardo Manzoni, Linda Ottoboni, Luca De Feo, Angelo Quattrini, Eloise Mastrangelo, Luca Sorre...