Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Research Project (R01)
Project #
3R01DK036729-10S1
Application #
2139859
Study Section
Biochemistry Study Section (BIO)
Project Start
1993-02-11
Project End
1995-12-31
Budget Start
1994-01-01
Budget End
1994-12-31
Support Year
10
Fiscal Year
1994
Total Cost
Indirect Cost
Name
Cincinnati Children's Hospital Medical Center
Department
Type
DUNS #
071284913
City
Cincinnati
State
OH
Country
United States
Zip Code
45229
Dai, Mei; Liou, Benjamin; Swope, Brittany et al. (2016) Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease. PLoS One 11:e0162367
Burrow, Thomas A; Sun, Ying; Prada, Carlos E et al. (2015) CNS, lung, and lymph node involvement in Gaucher disease type 3 after 11 years of therapy: clinical, histopathologic, and biochemical findings. Mol Genet Metab 114:233-241
Sun, Ying; Florer, Jane; Mayhew, Christopher N et al. (2015) Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology. PLoS One 10:e0118771
Kitatani, Kazuyuki; Wada, Masayuki; Perry, David et al. (2015) Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. PLoS One 10:e0136633
Dasgupta, Nupur; Xu, You-Hai; Li, Ronghua et al. (2015) Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model. Hum Mol Genet 24:7031-48
Liou, Benjamin; Haffey, Wendy D; Greis, Kenneth D et al. (2014) The LIMP-2/SCARB2 binding motif on acid ?-glucosidase: basic and applied implications for Gaucher disease and associated neurodegenerative diseases. J Biol Chem 289:30063-74
Pandey, Manoj Kumar; Jabre, Nicholas A; Xu, You-Hai et al. (2014) Gaucher disease: chemotactic factors and immunological cell invasion in a mouse model. Mol Genet Metab 111:163-71
Watson, Carey L; Mahe, Maxime M; MĂșnera, Jorge et al. (2014) An in vivo model of human small intestine using pluripotent stem cells. Nat Med 20:1310-4
Barnes, Sonya; Xu, You-Hai; Zhang, Wujuan et al. (2014) Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model. PLoS One 9:e116023
Xu, You-hai; Xu, Kui; Sun, Ying et al. (2014) Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice. Hum Mol Genet 23:3943-57

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