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435 results on '"Lysosomal Storage Diseases metabolism"'

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151. Activation of the Keap1/Nrf2 stress response pathway in autophagic vacuolar myopathies.

152. Stem Cell Therapy for the Central Nervous System in Lysosomal Storage Diseases.

153. Use of BODIPY-Cholesterol (TF-Chol) for Visualizing Lysosomal Cholesterol Accumulation.

154. Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders.

155. Clinical course of sly syndrome (mucopolysaccharidosis type VII).

156. Transcription factor EB: from master coordinator of lysosomal pathways to candidate therapeutic target in degenerative storage diseases.

157. Autophagy, lipophagy and lysosomal lipid storage disorders.

158. Mannose receptor-mediated delivery of moss-made α-galactosidase A efficiently corrects enzyme deficiency in Fabry mice.

159. A Comparative Study on the Alterations of Endocytic Pathways in Multiple Lysosomal Storage Disorders.

160. Lysosomal Acid Phosphatase Biosynthesis and Dysfunction: A Mini Review Focused on Lysosomal Enzyme Dysfunction in Brain.

161. Preface.

162. Carbohydrate-Processing Enzymes of the Lysosome: Diseases Caused by Misfolded Mutants and Sugar Mimetics as Correcting Pharmacological Chaperones.

163. Rosa Puertollano: The importance of recycling cellular trash.

164. [Chemical chaperone therapy for lysosomal storage diseases].

165. A CASQ1 founder mutation in three Italian families with protein aggregate myopathy and hyperCKaemia.

166. Loss of AP-5 results in accumulation of aberrant endolysosomes: defining a new type of lysosomal storage disease.

167. Property of lysosomal storage disease associated with midbrain pathology in the central nervous system of Lamp-2-deficient mice.

168. BK Channels Alleviate Lysosomal Storage Diseases by Providing Positive Feedback Regulation of Lysosomal Ca2+ Release.

169. Altered Clathrin-Independent Endocytosis in Type A Niemann-Pick Disease Cells and Rescue by ICAM-1-Targeted Enzyme Delivery.

170. Enhanced delivery of daidzein into fibroblasts and neuronal cells with cationic derivatives of gamma-cyclodextrin for the control of cellular glycosaminoglycans.

171. The clinical spectrum and pathophysiology of skeletal complications in lysosomal storage disorders.

172. Bridging the age spectrum of neurodegenerative storage diseases.

173. Commentary on some recent theses relevant to combating aging: February 2015.

174. Lysosomal storage diseases: from pathophysiology to therapy.

175. Combined Therapies for Lysosomal Storage Diseases.

176. Complex lipid trafficking in Niemann-Pick disease type C.

177. Quantifying storage material accumulation in tissue sections.

178. "Three sources and three component parts" of free oligosaccharides.

179. Lysosomal storage diseases and the heat shock response: convergences and therapeutic opportunities.

180. A mutation in the CASQ1 gene causes a vacuolar myopathy with accumulation of sarcoplasmic reticulum protein aggregates.

181. Impairment of homeostasis in lysosomal storage disorders.

182. The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases.

183. Sphingolipid lysosomal storage disorders.

184. Pharmacological chaperone therapy for lysosomal storage diseases.

185. Autophagy and human disease: emerging themes.

186. Sphingolipids and lysosomal pathologies.

187. Role of endosomes and lysosomes in human disease.

188. 2-Hydroxypropyl-β-cyclodextrin promotes transcription factor EB-mediated activation of autophagy: implications for therapy.

189. [The world of galactose and glucose: pathogenesis and therapy of lysosomal diseases].

190. Determination of the lysosomal hydrolase activity in blood collected on filter paper, an alternative to screen high risk populations.

191. Fast urinary screening of oligosaccharidoses by MALDI-TOF/TOF mass spectrometry.

192. A phenotypic compound screening assay for lysosomal storage diseases.

193. LAMP2 as a marker of EBV-mediated B lymphocyte transformation in the study of lysosomal storage diseases.

194. Metallothioneins as dynamic markers for brain disease in lysosomal disorders.

195. Murine neural stem cells model Hunter disease in vitro: glial cell-mediated neurodegeneration as a possible mechanism involved.

196. Lysosomal storage diseases--the horizon expands.

197. A somatic cell defect is associated with the onset of neurological symptoms in a lysosomal storage disease.

198. The lysosome: from waste bag to potential therapeutic target.

199. Glycobiology: progress, problems, and perspectives.

200. Animal models for lysosomal storage disorders.

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