A5050502741- Glycogen Storage Diseases and Myoclonus
- Biomedical Research and Pathophysiology
- Genetics and Neurodevelopmental Disorders
- Lysosomal Storage Disorders Research
- Amino Acid Enzymes and Metabolism
- Pancreatic function and diabetes
- Genetic and Kidney Cyst Diseases
- Diet, Metabolism, and Disease
- Liver Disease Diagnosis and Treatment
- Pancreatitis Pathology and Treatment
- Cancer, Hypoxia, and Metabolism
- Parathyroid Disorders and Treatments
- Neurogenetic and Muscular Disorders Research
- Diet and metabolism studies
- Diabetes Management and Research
- Endoplasmic Reticulum Stress and Disease
- Wnt/β-catenin signaling in development and cancer
- Cancer, Lipids, and Metabolism
- Carbohydrate Chemistry and Synthesis
- Diabetes Treatment and Management
- Renal Diseases and Glomerulopathies
- Metabolism, Diabetes, and Cancer
Inserm
2018-2024
Université Claude Bernard Lyon 1
2018-2024
Nutrition, Diabète et Cerveau
2019
Université de Lyon
2018
Ectopic lipid accumulation in the liver and kidneys is a hallmark of metabolic diseases leading to non-alcoholic fatty disease (NAFLD) chronic kidney (CKD). Moreover, recent data have highlighted strong correlation between NAFLD CKD incidences. In this study, we use two mouse models hepatic steatosis or CKD, each initiated independently other upon suppression glucose production specifically kidneys, elucidate mechanisms underlying development context NAFLD-like pathology. Mice with deletion...
Glycogen Storage Disease Type I (GSDI) is an inherited disease caused by glucose-6 phosphatase (G6Pase) deficiency, leading to a loss of endogenous glucose production and severe hypoglycemia. Moreover, most GSDI patients develop chronic kidney (CKD) due lipid accumulation in the kidney. Similar diabetic CKD, activation renin-angiotensin system (RAS) promotes renal fibrosis GSDI. Here, we investigated physiological molecular effects RAS blockers mice. A retrospective analysis function was...
Key Points Metabolism adaptations due to glucose-6 phosphate accumulation in glycogen storage disease type Ia kidneys, toward a Warburg-like metabolism, promoted cell proliferation. Metabolic perturbations directly affected primary cilium structure and cystogenesis kidneys. Background Glycogen (GSDIa) is rare metabolic disorder caused by mutations the catalytic subunit of phosphatase (G6PC1). This leads severe hypoglycemia, most young patients with GSDIa develop CKD. The kidney pathology...
The intra-peritoneal route was not sufficient for NPC elimination.Next, we compared the effects of each DT dose and found that kidney volume glomeruli per unit area were inversely related to (Figure 2).CONCLUSIONS: By establishing intra-amniotic injection is optimal administration DT, these results will facilitate studies regeneration in vivo.In addition, this method elimination mother's womb using iDTR system can only be applied regeneration, but also various disease models embryology research.
Mann-Whitney U test) in all nephropathy biopsies studied, especially the cytoplasm of distal tubular epithelial cells.