26 results on '"Cheng, Seng"'
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2. Basis for the relationship between Gaucher disease and synucleinopathies
3. AAV8-mediated expression of N-acetylglucosamine-1-phosphate transferase attenuates bone loss in a mouse model of mucolipidosis II.
4. Erythrocytes encapsulated with phenylalanine hydroxylase exhibit improved pharmacokinetics and lowered plasma phenylalanine levels in normal mice.
5. Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann–Pick C mice
6. IGF-1 delivery to CNS attenuates motor neuron cell death but does not improve motor function in type III SMA mice
7. Relationship between neuropathology and disease progression in the SOD1G93A ALS mouse
8. Inhibition of glycogen biosynthesis via mTORC1 suppression as an adjunct therapy for Pompe disease
9. Pulmonary delivery of recombinant acid sphingomyelinase improves clearance of lysosomal sphingomyelin from the lungs of a murine model of Niemann–Pick disease
10. Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann–Pick A disease
11. Hypoxia-inducible factor 1 mediates hypoxia-induced cardiomyocyte lipid accumulation by reducing the DNA binding activity of peroxisome proliferator-activated receptor α/retinoid X receptor
12. Intraparenchymal injections of acid sphingomyelinase results in regional correction of lysosomal storage pathology in the Niemann–Pick A mouse
13. A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease
14. Hyaluronidase increases the biodistribution of acid α-1,4 glucosidase in the muscle of Pompe disease mice: An approach to enhance the efficacy of enzyme replacement therapy
15. Differential effects of membrane and soluble Fas ligand on cardiomyocytes: role in ischemia/reperfusion injury
16. Hypoxia Up-regulates Expression of Peroxisome Proliferator-activated Receptor γ Angiopoietin-related Gene (PGAR) in Cardiomyocytes: Role of Hypoxia Inducible Factor 1α
17. Stabilization of Vascular Endothelial Growth Factor mRNA by Hypoxia-Inducible Factor 1
18. Tetrahydrobiopterin deficiency in the pathogenesis of Fabry disease.
19. Glucosylceramide synthase inhibition reduces α-synuclein pathology and improves cognition in murine models of synucleinopathy.
20. Evaluation of a novel substrate reduction therapy with CNS access in mouse models of neuronopathic Gaucher disease.
21. Antisense oligonucleotide-mediated suppression of muscle glycogen synthase 1 synthesis as an approach for substrate reduction therapy of Pompe disease.
22. A novel, selective and orally-available glucosylceramide synthase inhibitor for substrate reduction therapy of Fabry disease
23. Glucosylceramide Synthase Inhibition Reduces Gb3 and Lyso-Gb3 in a Mouse Model Of Fabry Disease
24. Inhibiting glycogen biosynthesis by mTORC1 suppression as an adjunct therapy for Pompe disease
25. 90. Efficacy of Genz-529468-mediated inhibition of glucosylceramide synthase in a mouse model of Sandhoff disease
26. 91. Management of lysosomal glycosphingolipid levels in animal models of Gaucher and Fabry disease with enzyme and substrate reduction therapies
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