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Mouse models of Tay–Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism
The Tay–Sachs and Sandhoff diseases are clinically similar neurodegenerative disorders and through disruption of the Hexa and Hexb genes in embryonic stem cells, mouse models corresponding to each disease are established. Expand
Reactive Oxygen Species Derived from NOX1/NADPH Oxidase Enhance Inflammatory Pain
It is indicated that NOX1/NADPH oxidase accelerates the translocation of PKCε in DRG neurons, thereby enhancing the TRPV1 activity and the sensitivity to painful stimuli. Expand
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder.
  • Y. Liu, R. Wada, +10 authors R. Proia
  • Biology, Medicine
  • The Journal of clinical investigation
  • 15 February 1999
The results support the validity of the substrate deprivation therapy and also highlight some limitations. Expand
Mice lacking both subunits of lysosomal β–hexosaminidase display gangliosidosis and mucopolysaccharidosis
It is demonstrated that glycosaminoglycans are crucial substrates for β- hexosaminidase and that their lack of storage in Tay-Sachs and Sandhoff diseases is due to functional redundancy in the β-hexosaminationidase enzyme system. Expand
Oxidized Galectin-1 Stimulates Macrophages to Promote Axonal Regeneration in Peripheral Nerves after Axotomy
It is proposed that axonal regeneration occurs in axotomized peripheral nerves as a result of cytosolic reduced galectin-1 being released from Schwann cells and injured axons, which then becomes oxidized in the extracellular space. Expand
Increase in intracellular Ca2+ and calcitonin gene-related peptide release through metabotropic P2Y receptors in rat dorsal root ganglion neurons
We examined the effects of the activation of metabotropic P2Y receptors on the intracellular Ca2+ concentration and the release of neuropeptide calcitonin gene-related peptide (CGRP) in isolatedExpand
SGLT2 Inhibitors as a Therapeutic Option for Diabetic Nephropathy
Renoprotection by SGLT2 inhibitors has been demonstrated in T2D patients with a high cardiovascular risk in randomized controlled trials (RCTs), and the favorable effects of SGLt2 inhibitors are explained by several potential mechanisms, including the attenuation of glomerular hyperfiltration, inflammation and oxidative stress. Expand
Motor Dysfunction in Type 5 Adenylyl Cyclase-null Mice*
It is implied that this isoform provides a site of convergence of both D1 and D2 dopaminergic signals and balances various motor functions and extends knowledge of the role of an effector enzyme isoform in regulating receptor signaling and neuronal functions. Expand
An in vitro model of the inhibition of axon growth in the lesion scar formed after central nervous system injury
An in vitro model of the fibrotic scar is developed using coculture of cerebral astrocytes and meningeal fibroblasts by adding transforming growth factor-beta1 (TGF- beta1), a potent fibrogenic factor and remarkably inhibited the neurite outgrowth of cerebellar neurons. Expand
Synthesis, localization and externalization of galectin‐1 in mature dorsal root ganglion neurons and Schwann cells
We recently confirmed that oxidized galectin‐1 is a novel factor enhancing axonal growth in peripheral nerves after axotomy, but the process of extracellular release and oxidization of endogenousExpand