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Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient miceAutophagy in lysosomal storage disordersThe cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionClarifying lysosomal storage diseasesImpairment of homeostasis in lysosomal storage disordersLysosomal storage diseases and the heat shock response: convergences and therapeutic opportunitiesThe lysosome: from waste bag to potential therapeutic targetCyclodextrin-mediated crystallization of acid β-glucosidase in complex with amphiphilic bicyclic nojirimycin analoguesStructure of sulfamidase provides insight into the molecular pathology of mucopolysaccharidosis IIIANew therapeutic approaches for Krabbe disease: The potential of pharmacological chaperonesLysosomal integral membrane protein-2: a new player in lysosome-related pathologyBoning up on autophagy: the role of autophagy in skeletal biologyClearance of apoptotic cells: implications in health and diseasehLGDB: a database of human lysosomal genes and their regulation.A lysosome-to-nucleus signalling mechanism senses and regulates the lysosome via mTOR and TFEBA tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies.Lysosomal exocytosis and lipid storage disorders.Lysosomal adaptation: how the lysosome responds to external cuesAberrant Ca2+ handling in lysosomal storage disordersBrain RNA-Seq Profiling of the Mucopolysaccharidosis Type II Mouse Model.Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases.Induced pluripotent stem cell models of lysosomal storage disorders.Lysosomal function in macromolecular homeostasis and bioenergetics in Parkinson's disease.Alteration of the proteostasis network of plant cells promotes the post-endoplasmic reticulum trafficking of recombinant mutant (L444P) human β-glucocerebrosidase.Guidelines for diagnosis and treatment of Hunter Syndrome for clinicians in Latin America.Expression of the disease on female carriers of X-linked lysosomal disorders: a brief review.Common and uncommon pathogenic cascades in lysosomal storage diseasesDysregulation of gene expression in a lysosomal storage disease varies between brain regions implicating unexpected mechanisms of neuropathology.Signals from the lysosome: a control centre for cellular clearance and energy metabolism.Differences in the predominance of lysosomal and autophagic pathologies between infants and adults with Pompe disease: implications for therapy.Lysosomal physiology.rnaset2 mutant zebrafish model familial cystic leukoencephalopathy and reveal a role for RNase T2 in degrading ribosomal RNA.Suppression of autophagy permits successful enzyme replacement therapy in a lysosomal storage disorder--murine Pompe disease.Progressive retinal degeneration and accumulation of autofluorescent lipopigments in Progranulin deficient mice.Arterial pathology in canine mucopolysaccharidosis-I and response to therapyLysosomal protein turnover contributes to the acquisition of TGFβ-1 induced invasive properties of mammary cancer cells.Purkinje cell compartmentation in the cerebellum of the lysosomal Acid phosphatase 2 mutant mouse (nax - naked-ataxia mutant mouse).TRPML: transporters of metals in lysosomes essential for cell survival?Pharmacological chaperone therapy for lysosomal storage diseases.Transcriptional activation of lysosomal exocytosis promotes cellular clearance.
P2860
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P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on 08 December 2008
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Lysosomal disorders: from storage to cellular damage.
@en
Lysosomal disorders: from storage to cellular damage.
@nl
type
label
Lysosomal disorders: from storage to cellular damage.
@en
Lysosomal disorders: from storage to cellular damage.
@nl
prefLabel
Lysosomal disorders: from storage to cellular damage.
@en
Lysosomal disorders: from storage to cellular damage.
@nl
P1476
Lysosomal disorders: from storage to cellular damage.
@en
P2093
Volkmar Gieselmann
P304
P356
10.1016/J.BBAMCR.2008.12.001
P407
P577
2008-12-08T00:00:00Z