Ablation of mouse phosphomannose isomerase (Mpi) causes mannose 6-phosphate accumulation, toxicity, and embryonic lethality
about
Metabolically programmed quality control system for dolichol-linked oligosaccharidesMetabolic manipulation of glycosylation disorders in humans and animal modelsMPI depletion enhances O-GlcNAcylation of p53 and suppresses the Warburg effect.The congenital disorders of glycosylation: a multifaceted group of syndromes.Mannose metabolism: more than meets the eye.Improvement of dolichol-linked oligosaccharide biosynthesis by the squalene synthase inhibitor zaragozic acid.Mannose efflux from the cells: a potential source of mannose in blood.Mannose-6-phosphate regulates destruction of lipid-linked oligosaccharidesUrinary tract effects of HPSE2 mutations.Mannose phosphate isomerase regulates fibroblast growth factor receptor family signaling and glioma radiosensitivity.Phosphomannose isomerase inhibitors improve N-glycosylation in selected phosphomannomutase-deficient fibroblasts.A zebrafish model of PMM2-CDG reveals altered neurogenesis and a substrate-accumulation mechanism for N-linked glycosylation deficiency.A zebrafish model of congenital disorders of glycosylation with phosphomannose isomerase deficiency reveals an early opportunity for corrective mannose supplementation.Understanding human glycosylation disorders: biochemistry leads the charge.Metabolic profiling reveals biochemical pathways and potential biomarkers associated with the pathogenesis of Krabbe disease.Towards a therapy for phosphomannomutase 2 deficiency, the defect in CDG-Ia patients.Mannose supplements induce embryonic lethality and blindness in phosphomannose isomerase hypomorphic mice.Mouse models for congenital disorders of glycosylation.Therapies and therapeutic approaches in Congenital Disorders of Glycosylation.Cloning, expression, purification, crystallization and preliminary X-ray crystallographic analysis of the mannose 6-phosphate isomerase from Salmonella typhimurium.Molecular imaging of N-linked glycosylation suggests glycan biosynthesis is a novel target for cancer therapy.Exogenous mannose does not raise steady state mannose-6-phosphate pools of normal or N-glycosylation-deficient human fibroblasts.Seizures and stupor during intravenous mannose therapy in a patient with CDG syndrome type 1b (MPI-CDG).The relative contribution of mannose salvage pathways to glycosylation in PMI-deficient mouse embryonic fibroblast cells.Increased litter size and super-ovulation rate in congenic C57BL mice carrying a polymorphic fragment of NFR/N origin at the Fecq4 locus of chromosome 9.CDG Therapies: From Bench to Bedside.
P2860
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P2860
Ablation of mouse phosphomannose isomerase (Mpi) causes mannose 6-phosphate accumulation, toxicity, and embryonic lethality
description
2006 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի մարտին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique Journal of Biological Chemistry
@fr
artículu científicu espublizáu en 2006
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im März 2006 veröffentlichter wissenschaftlicher Artikel
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scientific journal article
@en
vedecký článok (publikovaný 2006/03/03)
@sk
vědecký článek publikovaný v roce 2006
@cs
wetenschappelijk artikel (gepubliceerd op 2006/03/03)
@nl
наукова стаття, опублікована в березні 2006
@uk
name
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@ast
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@en
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@nl
type
label
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@ast
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@en
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@nl
prefLabel
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@ast
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@en
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@nl
P2093
P2860
P356
P1476
Ablation of mouse phosphomanno ...... icity, and embryonic lethality
@en
P2093
Alexander D Borowsky
Charles DeRossi
Erik A Eklund
Fangrong Zhang
Joseph A Davis
Vibeke Westphal
P2860
P304
P356
10.1074/JBC.M511982200
P407
P577
2005-12-08T00:00:00Z