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Results: 1-15 |
Results: 15
Cloning the beta-hexosaminidase genes
Authors: Proia, RL
Citation: Rl. Proia, Cloning the beta-hexosaminidase genes, ADV GENETIC, 44, 2001, pp. 127-135
Targeting the hexosaminidase genes: Mouse models of the G(M2) gangliosidoses
Authors: Proia, RL
Citation: Rl. Proia, Targeting the hexosaminidase genes: Mouse models of the G(M2) gangliosidoses, ADV GENETIC, 44, 2001, pp. 225-231
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures
Authors: Kawai, H Allende, ML Wada, R Kono, M Sango, K Deng, CX Miyakawa, T Crawley, JN Werth, N Bierfreund, U Sandhoff, K Proia, RL
Citation: H. Kawai et al., Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures, J BIOL CHEM, 276(10), 2001, pp. 6885-6888
Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation
Authors: Jeyakumar, M Norflus, F Tifft, CJ Cortina-Borja, M Butters, TD Proia, RL Perry, VH Dwek, RA Platt, FM
Citation: M. Jeyakumar et al., Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation, BLOOD, 97(1), 2001, pp. 327-329
Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse
Authors: Liu, YJ Wu, YP Wada, R Neufeld, EB Mullin, KA Howard, AC Pentchev, PG Vanier, MT Suzuki, K Proia, RL
Citation: Yj. Liu et al., Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse, HUM MOL GEN, 9(7), 2000, pp. 1087-1092
Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases
Authors: Tifft, CJ Proia, RL
Citation: Cj. Tifft et Rl. Proia, Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases, GLYCOBIOLOG, 10(12), 2000, pp. 1249-1258
Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency
Authors: Potratz, A Huttler, S Bierfreund, U Proia, RL Suzuki, K Sandhoff, K
Citation: A. Potratz et al., Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency, BBA-MOL BAS, 1502(3), 2000, pp. 391-397
Microglial activation precedes acute neurodegeneration in Sandhoff diseaseand is suppressed by bone marrow transplantation
Authors: Wada, R Tifft, CJ Proia, RL
Citation: R. Wada et al., Microglial activation precedes acute neurodegeneration in Sandhoff diseaseand is suppressed by bone marrow transplantation, P NAS US, 97(20), 2000, pp. 10954-10959
Edg-1, the G protein-coupled receptor for sphingosine-1-phosphate, is essential for vascular maturation
Authors: Liu, YJ Wada, R Yamashita, T Mi, YD Deng, CX Hobson, JP Rosenfeldt, HM Nava, VE Chae, SS Lee, MJ Liu, CH Hla, T Spiegel, S Proia, RL
Citation: Yj. Liu et al., Edg-1, the G protein-coupled receptor for sphingosine-1-phosphate, is essential for vascular maturation, J CLIN INV, 106(8), 2000, pp. 951-961
Distribution of enzyme-bearing cells in GM(2) gangliosidosis mice: regionally specific pattern of cellular infiltration following bone marrow transplantation
Authors: Oya, Y Proia, RL Norflus, F Tifft, CJ Langaman, C Suzuki, K
Citation: Y. Oya et al., Distribution of enzyme-bearing cells in GM(2) gangliosidosis mice: regionally specific pattern of cellular infiltration following bone marrow transplantation, ACT NEUROP, 99(2), 2000, pp. 161-168
A vital role for glycosphingolipid synthesis during development and differentiation
Authors: Yamashita, T Wada, R Sasaki, T Deng, CX Bierfreund, U Sandhoff, K Proia, RL
Citation: T. Yamashita et al., A vital role for glycosphingolipid synthesis during development and differentiation, P NAS US, 96(16), 1999, pp. 9142-9147
Mice lacking complex gangliosides develop Wallerian degeneration and myelination defects
Authors: Sheikh, KA Sun, J Liu, YJ Kawai, H Crawford, TO Proia, RL Griffin, JW Schnaar, RL
Citation: Ka. Sheikh et al., Mice lacking complex gangliosides develop Wallerian degeneration and myelination defects, P NAS US, 96(13), 1999, pp. 7532-7537
Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin
Authors: Jeyakumar, M Butters, TD Cortina-Borja, M Hunnam, V Proia, RL Perry, VH Dwek, RA Platt, FM
Citation: M. Jeyakumar et al., Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin, P NAS US, 96(11), 1999, pp. 6388-6393
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder
Authors: Liu, YJ Wada, R Kawai, H Sango, K Deng, CX Tai, T McDonald, MP Araujo, K Crawley, JN Bierfreund, U Sandhoff, K Suzuki, K Proia, RL
Citation: Yj. Liu et al., A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder, J CLIN INV, 103(4), 1999, pp. 497-505
Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice
Authors: Doering, T Proia, RL Sandhoff, K
Citation: T. Doering et al., Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice, FEBS LETTER, 447(2-3), 1999, pp. 167-170
Risultati: 1-15 |