173 results on '"Denis, Simone"'
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2. Tracer‐based lipidomics enables the discovery of disease‐specific candidate biomarkers in mitochondrial β‐oxidation disorders
3. Upregulation of the AMPK-FOXO1-PDK4 pathway is a primary mechanism of pyruvate dehydrogenase activity reduction and leads to increased glucose uptake in tafazzin-deficient cells
4. Upregulation of the AMPK-FOXO1-PDK4 pathway is a primary mechanism of pyruvate dehydrogenase activity reduction in tafazzin-deficient cells.
5. Human inborn errors of long-chain fatty acid oxidation show impaired inflammatory responses to TLR4-ligand LPS
6. Tracer-based lipidomics identifies novel disease-specific biomarkers in mitochondrial β-oxidation disorders
7. ACOX3 Dysfunction as a Potential Cause of Recurrent Spontaneous Vasospasm of Internal Carotid Artery
8. Barth syndrome cells display widespread remodeling of mitochondrial complexes without affecting metabolic flux distribution
9. Acute detachment of hexokinase II from mitochondria modestly increases oxygen consumption of the intact mouse heart
10. Lower Metabolic Potential and Impaired Metabolic Flexibility in Human Lymph Node Stromal Cells from Patients with Rheumatoid Arthritis
11. Neonatal Long-Chain 3-Ketoacyl-CoA Thiolase deficiency: Clinical-biochemical phenotype, sodium-D,L-3-hydroxybutyrate treatment experience and cardiac evaluation using speckle echocardiography
12. Clinical heterogeneity of mitochondrial NAD kinase deficiency caused by a NADK2 start loss variant
13. Pyruvate dehydrogenase complex plays a central role in brown adipocyte energy expenditure and fuel utilization during short-term beta-adrenergic activation
14. Lower Metabolic Potential and Impaired Metabolic Flexibility in Human Lymph Node Stromal Cells from Patients with Rheumatoid Arthritis.
15. Time‐restricted feeding during the inactive phase abolishes the daily rhythm in mitochondrial respiration in rat skeletal muscle
16. Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice
17. Inhibition of the neuromuscular acetylcholine receptor with atracurium activates FOXO/DAF‐16‐induced longevity
18. A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3
19. Mannose-Binding Lectin Is Required for the Effective Clearance of Apoptotic Cells by Adipose Tissue Macrophages During Obesity
20. Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia
21. Reduced nicotinamide mononucleotide is a new and potent NAD+precursor in mammalian cells and mice
22. Quantification of Myocardial Creatine and Triglyceride Content in the Human Heart: Precision and Accuracy of in vivo Proton Magnetic Resonance Spectroscopy
23. Detection of nonsterol isoprenoids by HPLC–MS/MS
24. Time‐restricted feeding during the inactive phase abolishes the daily rhythm in mitochondrial respiration in rat skeletal muscle.
25. Impaired amino acid metabolism contributes to fasting-induced hypoglycemia in fatty acid oxidation defects
26. Inhibition of the neuromuscular acetylcholine receptor with atracurium activates FOXO/DAF-16-induced longevity
27. Identification of an unusual variant peroxisome biogenesis disorder caused by mutations in the PEX16 gene
28. Clinical, Biochemical, and Mutational Spectrum of Peroxisomal Acyl-Coenzyme A Oxidase Deficiency
29. Defective peroxisomal catabolism of branched fatty acyl coenzyme A in mice lacking the sterol carrier protein-2/sterol carrier protein-x gene function
30. Peroxisomal Branched Chain Fatty Acid β-Oxidation Pathway Is Upregulated in Prostate Cancer
31. Cardiolipin-induced activation of pyruvate dehydrogenase links mitochondrial lipid biosynthesis to TCA cycle function
32. A Defective Pentose Phosphate Pathway Reduces Inflammatory Macrophage Responses during Hypercholesterolemia
33. Increased cardiac fatty acid oxidation in a mouse model with decreased malonyl-CoA sensitivity of CPT1B
34. Mitochondrial disruption in peroxisome deficient cells is hepatocyte selective but is not mediated by common hepatic peroxisomal metabolites
35. A novel case of ACOX2 deficiency leads to recognition of a third human peroxisomal acyl-CoA oxidase
36. Increased cardiac fatty acid oxidation in a mouse model with decreased malonyl-CoA sensitivity of CPT1B.
37. Clinical heterogeneity of mitochondrial NAD kinase deficiency caused by a <italic>NADK2</italic> start loss variant.
38. A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3
39. Alpha-methylacyl-CoA racemase as an androgen-independent growth modifier in prostate cancer
40. Genetic basis of hyperlysinemia
41. Peroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids
42. Fasting-Induced Myocardial Lipid Accumulation in Long-Chain Acyl-CoA Dehydrogenase Knockout Mice Is Accompanied by Impaired Left Ventricular Function
43. Bile acids: the role of peroxisomes
44. Toxicity of peroxisomal C27-bile acid intermediates
45. Corrigendum to “Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophy” [Mol. Genet. Metab. 84 (2005) 144–151]
46. Design, Synthesis, and In Vitro Testing of α-Methylacyl-CoA Racemase Inhibitors
47. Developmental Changes of Bile Acid Composition and Conjugation in L- and D-Bifunctional Protein Single and Double Knockout Mice
48. Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophy
49. Clinical and biochemical spectrum of D-bifunctional protein deficiency
50. Peroxisomal branched chain fatty acid ?-oxidation pathway is upregulated in prostate cancer
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