Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase-beta-deficient neutrophils in a congenital neutropenia syndrome
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JAGN1 deficiency causes aberrant myeloid cell homeostasis and congenital neutropeniaFunctional analysis of mutations in a severe congenital neutropenia syndrome caused by glucose-6-phosphatase-β deficiencyG-CSF improves murine G6PC3-deficient neutrophil function by modulating apoptosis and energy homeostasisThe SLC37 family of sugar-phosphate/phosphate exchangersCongenital neutropenia: diagnosis, molecular bases and patient management.A novel homozygous mutation in G6PC3 presenting as cyclic neutropenia and severe congenital neutropenia in the same family.Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib.Coronary artery endothelial transcriptome in vivo: identification of endoplasmic reticulum stress and enhanced reactive oxygen species by gene connectivity network analysis.Glucose-6-phosphatase-β, implicated in a congenital neutropenia syndrome, is essential for macrophage energy homeostasis and functionality.Quantitative proteomic analysis of Niemann-Pick disease, type C1 cerebellum identifies protein biomarkers and provides pathological insight.The SLC37 family of phosphate-linked sugar phosphate antiporters.Neutropenia in primary immunodeficiencyHepatic mitochondrial dysfunction is a feature of Glycogen Storage Disease Type Ia (GSDIa).Inborn errors of metabolism underlying primary immunodeficiencies.Type I glycogen storage diseases: disorders of the glucose-6-phosphatase/glucose-6-phosphate transporter complexes.Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics.Transforming growth factor-β1 regulates the nascent hematopoietic stem cell niche by promoting gluconeogenesis.Survival and differentiation defects contribute to neutropenia in glucose-6-phosphatase-β (G6PC3) deficiency in a model of mouse neutrophil granulocyte differentiation.Glycolysis regulates the expansion of myeloid-derived suppressor cells in tumor-bearing hosts through prevention of ROS-mediated apoptosis.Liver-directed gene therapy for murine glycogen storage disease type Ib.Glucose-6-Phosphatase Catalytic Subunit 3 (G6PC3) Deficiency Associated With Autoinflammatory Complications.Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia.Inflammatory bowel disease and T cell lymphopenia in G6PC3 deficiency.The Physiopathological Role of the Exchangers Belonging to the SLC37 Family.Effective Glucose Uptake by Human Astrocytes Requires Its Sequestration in the Endoplasmic Reticulum by Glucose-6-Phosphatase-β
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P2860
Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase-beta-deficient neutrophils in a congenital neutropenia syndrome
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2010 nî lūn-bûn
@nan
2010 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@ast
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@en
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@nl
type
label
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@ast
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@en
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@nl
prefLabel
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@ast
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@en
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@nl
P2093
P2860
P50
P921
P1433
P1476
Lack of glucose recycling betw ...... ongenital neutropenia syndrome
@en
P2093
Hyun Sik Jun
Janice Y Chou
Suk See De Ravin
Young Mok Lee
Yuk Yin Cheung
P2860
P304
P356
10.1182/BLOOD-2009-12-258491
P407
P577
2010-10-14T00:00:00Z