Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. - Wikidata - wikimedia - TriplyDB

Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.

Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.

http://www.wikidata.org/entity/Q34791322

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Acid beta-glucosidase 1 counteracts p38delta-dependent induction of interleukin-6: possible role for ceramide as an anti-inflammatory lipid Glucocerebrosidase is shaking up the synucleinopathies Clinical manifestations and management of Gaucher disease Gaucher disease and malignancy: a model for cancer pathogenesis in an inborn error of metabolism Immunoglobulin and free light chain abnormalities in Gaucher disease type I: data from an adult cohort of 63 patients and review of the literature.Effective cell and gene therapy in a murine model of Gaucher disease.Dysregulated sphingolipid metabolism in endometriosis One Year Follow-Up Risk Assessment in SKH-1 Mice and Wounds Treated with an Argon Plasma Jet.The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase.Expanding spectrum of the association between Type 1 Gaucher disease and cancers: a series of patients with up to 3 sequential cancers of multiple types--correlation with genotype and phenotype.Hyperferritinemia and iron overload in type 1 Gaucher disease.Glucocerebrosidase gene-deficient mouse recapitulates Gaucher disease displaying cellular and molecular dysregulation beyond the macrophage.Neuronopathic Gaucher's disease: induced pluripotent stem cells for disease modelling and testing chaperone activity of small compounds.Augmentation of phenotype in a transgenic Parkinson mouse heterozygous for a Gaucher mutation Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders.Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses.Substrate reduction therapy: miglustat as a remedy for symptomatic patients with Gaucher disease type 1.Type II NKT-TFH cells against Gaucher lipids regulate B-cell immunity and inflammation Future perspectives for glycolipid research in medicine.Ceramides as modulators of cellular and whole-body metabolism Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.Systemic inflammation and neurodegeneration in a mouse model of multiple sulfatase deficiency Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease Gaucher disease gene GBA functions in immune regulation.Lysosomal storage and impaired autophagy lead to inflammasome activation in Gaucher macrophages.Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis.Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice.Upregulation of proinflammatory cytokines in the fetal brain of the Gaucher mouse.Enzyme therapy for the treatment of type 1 Gaucher disease: clinical outcomes and dose - response relationships.Glucocerebrosidase 2 gene deletion rescues type 1 Gaucher disease.Animal models for Gaucher disease research.Animal models for metabolic, neuromuscular and ophthalmological rare diseases.The role of inflammation in sporadic and familial Parkinson's disease.Glucosylsphingosine promotes α-synuclein pathology in mutant GBA-associated Parkinson's disease.Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance.Coenzyme Q10 partially restores pathological alterations in a macrophage model of Gaucher disease.Gaucher disease: Progress and ongoing challenges.Induced Pluripotent Stem Cell Modeling of Gaucher's Disease: What Have We Learned?Intracellular metabolite β-glucosylceramide is an endogenous Mincle ligand possessing immunostimulatory activity.

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Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.

http://www.wikidata.org/entity/Q34791322

description

2002 nî lūn-bûn

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2002 թուականի Մայիսին հրատարակուած գիտական յօդուած

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2002 թվականի մայիսին հրատարակված գիտական հոդված

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2002年の論文

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2002年論文

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2002年論文

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2002年論文

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2002年論文

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2002年论文

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Systemic inflammation in gluco ...... imal glucosylceramide storage.

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Systemic inflammation in gluco ...... imal glucosylceramide storage.

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Systemic inflammation in gluco ...... imal glucosylceramide storage.

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Systemic inflammation in gluco ...... imal glucosylceramide storage.

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Journal of Clinical Investigation

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Systemic inflammation in gluco ...... imal glucosylceramide storage.

@en

P2093

Hiroki Mizukami

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Konrad Sandhoff

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Mari Kono

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Norbert Werth

http://www.w3.org/2001/XMLSchema#string

Richard L Proia

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Roger Sandhoff

http://www.w3.org/2001/XMLSchema#string

Ryuichi Wada

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Tadashi Yamashita

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Yide Mi

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Yujing Liu

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P2860

Identification of a molecular target of psychosine and its role in globoid cell formation.

Mice lacking the orphan G protein-coupled receptor G2A develop a late-onset autoimmune syndrome

Consequences of beta-glucocerebrosidase deficiency in epidermis. Ultrastructure and permeability barrier alterations in Gaucher disease

Systemic AL amyloidosis in Gaucher disease. A case report and review of the literature

Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure

Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis

A vital role for glycosphingolipid synthesis during development and differentiation

Pathologic gene expression in Gaucher disease: up-regulation of cysteine proteinases including osteoclastic cathepsin K.

RETRACTED: Lysophosphatidylcholine as a ligand for the immunoregulatory receptor G2A.

Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases.

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1215-1221

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10.1172/JCI0214530

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9

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109

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2002-05-01T00:00:00Z

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11994410

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150961

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