about
Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progressionNiemann-Pick disease type CAng2/fat-free is a conserved subunit of the Golgi-associated retrograde protein complexA key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolismThe cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionImpairment of homeostasis in lysosomal storage disordersLysosomal storage diseases and the heat shock response: convergences and therapeutic opportunitiesAtaxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).A canine Arylsulfatase G (ARSG) mutation leading to a sulfatase deficiency is associated with neuronal ceroid lipofuscinosis.Impaired lysosomal trimming of N-linked oligosaccharides leads to hyperglycosylation of native lysosomal proteins in mice with alpha-mannosidosis.Lysosomal exocytosis and lipid storage disorders.The factors affecting lipid profile in adult patients with MucopolysaccharidosisGenistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic diseaseMulti-system disorders of glycosphingolipid and ganglioside metabolismLysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders.Genetic and pathological links between Parkinson's disease and the lysosomal disorder Sanfilippo syndrome.Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIBLysosomal physiology.Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.Defective macroautophagic turnover of brain lipids in the TgCRND8 Alzheimer mouse model: prevention by correcting lysosomal proteolytic deficits.Secondary storage of dermatan sulfate in Sanfilippo disease.Manipulating ionic strength to improve single cell electrophoretic separationsEndosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.Ganglioside biochemistryBis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses.X-linked Angelman-like syndrome caused by Slc9a6 knockout in mice exhibits evidence of endosomal-lysosomal dysfunction.Loss of AP-5 results in accumulation of aberrant endolysosomes: defining a new type of lysosomal storage diseaseIn situ detection of GM1 and GM2 gangliosides using immunohistochemical and immunofluorescent techniques for auxiliary diagnosis of canine and feline gangliosidoses.Multimodal detection of GM2 and GM3 lipid species in the brain of mucopolysaccharidosis type II mouse by serial imaging mass spectrometry and immunohistochemistry.Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice.Neuraminidase-1 contributes significantly to the degradation of neuronal B-series gangliosides but not to the bypass of the catabolic block in Tay-Sachs mouse models.Different molecular mechanisms involved in spontaneous and oxidative stress-induced mitochondrial fragmentation in tripeptidyl peptidase-1 (TPP-1)-deficient fibroblasts.A Drosophila Genome-Wide Screen Identifies Regulators of Steroid Hormone Production and Developmental TimingA Christianson syndrome-linked deletion mutation (∆(287)ES(288)) in SLC9A6 disrupts recycling endosomal function and elicits neurodegeneration and cell deathA Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts.Sterols and sphingolipids: dynamic duo or partners in crime?Lysosomal storage disease: revealing lysosomal function and physiology.Pathology and current treatment of neurodegenerative sphingolipidoses.Myelin lesions associated with lysosomal and peroxisomal disorders.Epilepsy in children--when should we think neurometabolic disease?
P2860
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P2860
description
2008 nî lūn-bûn
@nan
2008 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Secondary lipid accumulation in lysosomal disease.
@ast
Secondary lipid accumulation in lysosomal disease.
@en
type
label
Secondary lipid accumulation in lysosomal disease.
@ast
Secondary lipid accumulation in lysosomal disease.
@en
prefLabel
Secondary lipid accumulation in lysosomal disease.
@ast
Secondary lipid accumulation in lysosomal disease.
@en
P2860
P1476
Secondary lipid accumulation in lysosomal disease.
@en
P2093
Marie T Vanier
Steven U Walkley
P2860
P304
P356
10.1016/J.BBAMCR.2008.11.014
P407
P577
2008-12-09T00:00:00Z