-
1
-
-
0037248733
-
Renoprotection: Clues from knockout models of rare diseases
-
Abbate M, Remuzzi G: Renoprotection: Clues from knockout models of rare diseases. Kidney Int 63:764-766 (2003).
-
(2003)
Kidney Int
, vol.63
, pp. 764-766
-
-
Abbate, M.1
Remuzzi, G.2
-
2
-
-
0036174534
-
Quantitative trait loci influence renal disease progression in a mouse model of Alport syndrome
-
Andrews KL, Mudd JL, Li C, Miner JH: Quantitative trait loci influence renal disease progression in a mouse model of Alport syndrome. Am J Pathol 160:721-730 (2002).
-
(2002)
Am J Pathol
, vol.160
, pp. 721-730
-
-
Andrews, K.L.1
Mudd, J.L.2
Li, C.3
Miner, J.H.4
-
3
-
-
0025292712
-
Identification of mutations in the COL4A5 collagen gene in Alport syndrome
-
Barker DE, Hostikka SL, Zhou J, Show LT, Oliphant AR, Gerken SC, Gregory MC, Skolnick MH, Atkin CL, Tryggvason K: Identification of mutations in the COL4A5 collagen gene in Alport syndrome. Science 248:1224-1227 (1990).
-
(1990)
Science
, vol.248
, pp. 1224-1227
-
-
Barker, D.E.1
Hostikka, S.L.2
Zhou, J.3
Show, L.T.4
Oliphant, A.R.5
Gerken, S.C.6
Gregory, M.C.7
Skolnick, M.H.8
Atkin, C.L.9
Tryggvason, K.10
-
5
-
-
0006275388
-
Human alpha-galactosidase A: Nucleotide sequence of a cDNA clone encoding the mature enzyme
-
Bishop DF, Calhoun DH, Bernstein HS, Hantzopoulos P, Quinn M, Desnick RJ: Human alpha-galactosidase A: nucleotide sequence of a cDNA clone encoding the mature enzyme. Proc Natl Acad Sci USA 83:4859-4863 (1986).
-
(1986)
Proc Natl Acad Sci USA
, vol.83
, pp. 4859-4863
-
-
Bishop, D.F.1
Calhoun, D.H.2
Bernstein, H.S.3
Hantzopoulos, P.4
Quinn, M.5
Desnick, R.J.6
-
6
-
-
0041800664
-
Structural organization of the human alpha-galactosidase A gene: Further evidence for the absence of a 3′ untranslated region
-
Bishop DF, Kornreich R, Desnick RJ: Structural organization of the human alpha-galactosidase A gene: further evidence for the absence of a 3′ untranslated region. Proc Natl Acad Sci USA 85:3903-3907 (1988).
-
(1988)
Proc Natl Acad Sci USA
, vol.85
, pp. 3903-3907
-
-
Bishop, D.F.1
Kornreich, R.2
Desnick, R.J.3
-
7
-
-
0014216741
-
Enzymatic defect in Fabry's disease: Ceramidetrihexosidase deficiency
-
Brady RO, Gal AE, Bradley RM, Martensson E, Warshaw AL, Laster L: Enzymatic defect in Fabry's disease: Ceramidetrihexosidase deficiency. New Engl J Med 276:1163-1167 (1967).
-
(1967)
New Engl J Med
, vol.276
, pp. 1163-1167
-
-
Brady, R.O.1
Gal, A.E.2
Bradley, R.M.3
Martensson, E.4
Warshaw, A.L.5
Laster, L.6
-
8
-
-
0037406311
-
Fabry disease: Diagnosis and treatment
-
Breunig F, Weidemann F, Beer M, Eggert A, Krane V, Spindler M, Sandstede J, Strotmann J, Wanner C: Fabry disease: Diagnosis and treatment. Kidney Int 63:S181-S185 (2003a).
-
(2003)
Kidney Int
, vol.63
-
-
Breunig, F.1
Weidemann, F.2
Beer, M.3
Eggert, A.4
Krane, V.5
Spindler, M.6
Sandstede, J.7
Strotmann, J.8
Wanner, C.9
-
9
-
-
0042833200
-
Enzyme replacement therapy in Fabry disease: Clinical implications
-
Breunig F, Knoll A, Wanner C: Enzyme replacement therapy in Fabry disease: clinical implications. Curr Opin Nephrol Hypertens 12:491-495 (2003b).
-
(2003)
Curr Opin Nephrol Hypertens
, vol.12
, pp. 491-495
-
-
Breunig, F.1
Knoll, A.2
Wanner, C.3
-
10
-
-
0032986645
-
Long-term effects of cyclosporine A in Alport's syndrome
-
Callis I, Vila A, Carrera M: Long-term effects of cyclosporine A in Alport's syndrome. Kidney Int 55:1051-1056 (1999).
-
(1999)
Kidney Int
, vol.55
, pp. 1051-1056
-
-
Callis, I.1
Vila, A.2
Carrera, M.3
-
11
-
-
0030944041
-
Mycophenolate mofetil limits renal damage and prolongs life in murine lupus autoimmune disease
-
Corna D, Morigi M, Facchinetti D, Bertani T, Zoja C, Remuzzi G: Mycophenolate mofetil limits renal damage and prolongs life in murine lupus autoimmune disease. Kidney Int 51:1583-1589 (1997).
-
(1997)
Kidney Int
, vol.51
, pp. 1583-1589
-
-
Corna, D.1
Morigi, M.2
Facchinetti, D.3
Bertani, T.4
Zoja, C.5
Remuzzi, G.6
-
12
-
-
0029829425
-
Collagen COL4A3 knockout: A mouse model for autosomal Alport syndrome
-
Cosgrove D, Meehan DT, Grunkemeyer JA, Kornak JM, Sayers R, Hunter WJ, Samuelson GC: Collagen COL4A3 knockout: A mouse model for autosomal Alport syndrome. Genes Dev 10:2981-2992 (1996).
-
(1996)
Genes Dev
, vol.10
, pp. 2981-2992
-
-
Cosgrove, D.1
Meehan, D.T.2
Grunkemeyer, J.A.3
Kornak, J.M.4
Sayers, R.5
Hunter, W.J.6
Samuelson, G.C.7
-
13
-
-
0033623532
-
Integrin α1β1 and transforming growth factor-β1 play distinct roles in Alport glomerular pathogenesis and serve as dual targets for metabolic therapy
-
Cosgrove D, Rodgers K, Meehan D, Miller C, Bovard K, Gilroy A, Gardner H, Kotelianski V, Gotwals P, Amatucci A, Kalluri R: Integrin α1β1 and transforming growth factor-β1 play distinct roles in Alport glomerular pathogenesis and serve as dual targets for metabolic therapy. Am J Pathol 157:1649-1659 (2000).
-
(2000)
Am J Pathol
, vol.157
, pp. 1649-1659
-
-
Cosgrove, D.1
Rodgers, K.2
Meehan, D.3
Miller, C.4
Bovard, K.5
Gilroy, A.6
Gardner, H.7
Kotelianski, V.8
Gotwals, P.9
Amatucci, A.10
Kalluri, R.11
-
14
-
-
0035097499
-
A phase 1/2 clinical trial of enzyme replacement in Fabry disease: Pharmacokinetic, substrate clearance, and safety studies
-
Eng CM, Banikazemi M, Gordon RE, Goldman M, Phelps R, Kim L, Gass A, Winston J, Dikman S, Fallon JT, Brodie S, Stacy CB, Mehta D, Parsons R, Norton K, O'Callaghan M, Desnick RJ: A phase 1/2 clinical trial of enzyme replacement in Fabry disease: Pharmacokinetic, substrate clearance, and safety studies. Am J Hum Genet 68:711-722 (2001).
-
(2001)
Am J Hum Genet
, vol.68
, pp. 711-722
-
-
Eng, C.M.1
Banikazemi, M.2
Gordon, R.E.3
Goldman, M.4
Phelps, R.5
Kim, L.6
Gass, A.7
Winston, J.8
Dikman, S.9
Fallon, J.T.10
Brodie, S.11
Stacy, C.B.12
Mehta, D.13
Parsons, R.14
Norton, K.15
O'Callaghan, M.16
Desnick, R.J.17
-
15
-
-
0032913758
-
Relevance of systemic lupus erythematosus nephritis animal models to human disease
-
Foster MH: Relevance of systemic lupus erythematosus nephritis animal models to human disease. Semin Nephrol 19:12-24 (1999).
-
(1999)
Semin Nephrol
, vol.19
, pp. 12-24
-
-
Foster, M.H.1
-
16
-
-
0032999536
-
Lupus nephritis: Update on pathogenesis and disease mechanisms
-
Foster MH, Kelley VR: Lupus nephritis: Update on pathogenesis and disease mechanisms. Semin Nephrol 19:173-181 (1999).
-
(1999)
Semin Nephrol
, vol.19
, pp. 173-181
-
-
Foster, M.H.1
Kelley, V.R.2
-
17
-
-
0037248954
-
Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome
-
Gross O, Beirowski B, Keopke M-L, Kuck J, Reiner M, Addicks K, Smyth N, Schulze-Lohoff E, Weber M: Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome. Kidney Int 63:438-446 (2003).
-
(2003)
Kidney Int
, vol.63
, pp. 438-446
-
-
Gross, O.1
Beirowski, B.2
Keopke, M.-L.3
Kuck, J.4
Reiner, M.5
Addicks, K.6
Smyth, N.7
Schulze-Lohoff, E.8
Weber, M.9
-
18
-
-
0028906290
-
The severe perinatal form of autosomal recessive polycystic kidney disease (ARPKD) maps to chromosome 6p21.1→p12: Implications for genetic counseling
-
Guay-Woodford L, Muecher G, Hopkins S, Avner E, Germino G, Guillot A, Herrin J, Holleman R, Irons D, Primack W, Thomson P, Waldo F, Lunt P, Zerres K: The severe perinatal form of autosomal recessive polycystic kidney disease (ARPKD) maps to chromosome 6p21.1→p12: Implications for genetic counseling. Am J Hum Genet 56:1101-1107 (1995).
-
(1995)
Am J Hum Genet
, vol.56
, pp. 1101-1107
-
-
Guay-Woodford, L.1
Muecher, G.2
Hopkins, S.3
Avner, E.4
Germino, G.5
Guillot, A.6
Herrin, J.7
Holleman, R.8
Irons, D.9
Primack, W.10
Thomson, P.11
Waldo, F.12
Lunt, P.13
Zerres, K.14
-
19
-
-
0034130222
-
Quantitative trait loci modulate renal cystic disease severity in the mouse bpk model
-
Guay-Woodford LM, Wright CJ, Walz G, Churchill GA: Quantitative trait loci modulate renal cystic disease severity in the mouse bpk model. J Am Soc Nephrol 11:1253-1260 (2000).
-
(2000)
J Am Soc Nephrol
, vol.11
, pp. 1253-1260
-
-
Guay-Woodford, L.M.1
Wright, C.J.2
Walz, G.3
Churchill, G.A.4
-
20
-
-
0141648351
-
A human-mouse chimera of the alpha3alpha4alpha5(IV) collagen promoter rescues the renal phenotype in Col4a3-/- Alport mice
-
Heidet L, Borza DB, Jouin M, Sich M, Mattei MG, Sado Y, Hudson BG, Hastie N, Antignac C, Gubler M-C: A human-mouse chimera of the alpha3alpha4alpha5( IV) collagen promoter rescues the renal phenotype in Col4a3-/- Alport mice. Am J Pathol 163:1633-1644 (2003).
-
(2003)
Am J Pathol
, vol.163
, pp. 1633-1644
-
-
Heidet, L.1
Borza, D.B.2
Jouin, M.3
Sich, M.4
Mattei, M.G.5
Sado, Y.6
Hudson, B.G.7
Hastie, N.8
Antignac, C.9
Gubler, M.-C.10
-
21
-
-
0033966792
-
Animal models of Alport syndrome: Advancing the prospects for effective human gene therapy
-
Heikkila P, Tryggvason K, Thorner P: Animal models of Alport syndrome: advancing the prospects for effective human gene therapy. Exp Nephrol 8:1.7 (2000).
-
(2000)
Exp Nephrol
, vol.8
, pp. 1-7
-
-
Heikkila, P.1
Tryggvason, K.2
Thorner, P.3
-
22
-
-
0034973112
-
Adenovirus-mediated transfer of type IV collagen alpha 5 chain cDNA into swine kidney in vivo: Deposition of the protein into the glomerular basement membrane
-
Heikkila P, Tibell A, Morita T, Chen Y, Wu G, Sado Y, Ninomiya Y, Pettersson E, Tryggvason K: Adenovirus-mediated transfer of type IV collagen alpha 5 chain cDNA into swine kidney in vivo: deposition of the protein into the glomerular basement membrane. Gene Therapy 8:882-890 (2001).
-
(2001)
Gene Therapy
, vol.8
, pp. 882-890
-
-
Heikkila, P.1
Tibell, A.2
Morita, T.3
Chen, Y.4
Wu, G.5
Sado, Y.6
Ninomiya, Y.7
Pettersson, E.8
Tryggvason, K.9
-
23
-
-
0014376492
-
The immunology and pathology of NZB mice
-
Howie JB, Helyer BJ: The immunology and pathology of NZB mice. Adv Immunol 9:215-266 (1968).
-
(1968)
Adv Immunol
, vol.9
, pp. 215-266
-
-
Howie, J.B.1
Helyer, B.J.2
-
24
-
-
0038469583
-
Alport's syndrome, Goodpasture's syndrome, and type IV collagen
-
Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG: Alport's syndrome, Goodpasture's syndrome, and type IV collagen. New Engl J Med 348:2543-2556 (2003).
-
(2003)
New Engl J Med
, vol.348
, pp. 2543-2556
-
-
Hudson, B.G.1
Tryggvason, K.2
Sundaramoorthy, M.3
Neilson, E.G.4
-
25
-
-
0028917291
-
Localization of a murine recessive polycystic kidney disease mutation and modifying loci that affect disease severity
-
Iakoubova O, Dunshkin H, Beier D: Localization of a murine recessive polycystic kidney disease mutation and modifying loci that affect disease severity. Genomics 26:107-114 (1995).
-
(1995)
Genomics
, vol.26
, pp. 107-114
-
-
Iakoubova, O.1
Dunshkin, H.2
Beier, D.3
-
26
-
-
0035163539
-
Fabry disease: Preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice
-
Ioannou YA, Zeidner KM, Gordon RE, Desnick RJ: Fabry disease: Preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice. Am J Hum Genet 68:14-25 (2001).
-
(2001)
Am J Hum Genet
, vol.68
, pp. 14-25
-
-
Ioannou, Y.A.1
Zeidner, K.M.2
Gordon, R.E.3
Desnick, R.J.4
-
27
-
-
0035956882
-
Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice
-
Jung S-C, Han IH, Limaye A, Xu R, Gelderman MP, Zerfas P, Tirumalai K, Murray GJ, During MJ, Brady RO, Qasba P: Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice. Proc Natl Acad Sci USA 98:2676-2681 (2001).
-
(2001)
Proc Natl Acad Sci USA
, vol.98
, pp. 2676-2681
-
-
Jung, S.-C.1
Han, I.H.2
Limaye, A.3
Xu, R.4
Gelderman, M.P.5
Zerfas, P.6
Tirumalai, K.7
Murray, G.J.8
During, M.J.9
Brady, R.O.10
Qasba, P.11
-
28
-
-
0034652247
-
Polycystin 1 is required for the structural integrity of blood vessels
-
Kim K, Drummond I, Ibraghimov-Beskrovnaya O, Klinger K, Arnaout MA: Polycystin 1 is required for the structural integrity of blood vessels. Proc Natl Acad Sci USA 97:1731-1736 (2000).
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 1731-1736
-
-
Kim, K.1
Drummond, I.2
Ibraghimov-Beskrovnaya, O.3
Klinger, K.4
Arnaout, M.A.5
-
29
-
-
0033942181
-
Treatment of murine lupus with cDNA encoding IFN-gamma R/Fc
-
Lawson BR, Prud'homme GJ, Chang Y, Gardner HA, Kuan J, Kono DH, Theofilopoulos AN: Treatment of murine lupus with cDNA encoding IFN-gamma R/Fc. J Clin Invest 106:207-215 (2000).
-
(2000)
J Clin Invest
, vol.106
, pp. 207-215
-
-
Lawson, B.R.1
Prud'homme, G.J.2
Chang, Y.3
Gardner, H.A.4
Kuan, J.5
Kono, D.H.6
Theofilopoulos, A.N.7
-
30
-
-
0028069132
-
Mutations in the type IV collagen α3 (COL4A3) gene in autosomal recessive Alport syndrome
-
Lemmink HH, Mochizuki T, van de Heuvel LPWJ, Schroder CH, Barrientos A, Monnens LAH, van Oost BA, Brunner HG, Reeders ST, Smeets HJM: Mutations in the type IV collagen α3 (COL4A3) gene in autosomal recessive Alport syndrome. Hum Mol Genet 3:1269-1273 (1994).
-
(1994)
Hum Mol Genet
, vol.3
, pp. 1269-1273
-
-
Lemmink, H.H.1
Mochizuki, T.2
Van De Heuvel, L.P.W.J.3
Schroder, C.H.4
Barrientos, A.5
Monnens, L.A.H.6
Van Oost, B.A.7
Brunner, H.G.8
Reeders, S.T.9
Smeets, H.J.M.10
-
31
-
-
0031252295
-
Perinatal lethality with kidney and pancreas defects in mice with a targetted Pkd1 mutation
-
Lu W, Peissel B, Babakhanlou H, Pavlova A, Geng L, Fan X, Larson C, Brent G, Zhou J: Perinatal lethality with kidney and pancreas defects in mice with a targetted Pkd1 mutation. Nat Genet 17:179-181 (1997).
-
(1997)
Nat Genet
, vol.17
, pp. 179-181
-
-
Lu, W.1
Peissel, B.2
Babakhanlou, H.3
Pavlova, A.4
Geng, L.5
Fan, X.6
Larson, C.7
Brent, G.8
Zhou, J.9
-
32
-
-
0032909095
-
Late onset of renal and hepatic cysts in Pkd1-targeted heterozygotes
-
Lu W, Fan X, Basora N, Babakhanlou H, Law T, Rifai N, Harris PC, Perez-Atayde AR, Rennke HG, Zhou J: Late onset of renal and hepatic cysts in Pkd1-targeted heterozygotes. Nat Genet 21:160-161 (1999a).
-
(1999)
Nat Genet
, vol.21
, pp. 160-161
-
-
Lu, W.1
Fan, X.2
Basora, N.3
Babakhanlou, H.4
Law, T.5
Rifai, N.6
Harris, P.C.7
Perez-Atayde, A.R.8
Rennke, H.G.9
Zhou, J.10
-
33
-
-
0033569491
-
Insertional mutation of the collagen genes Col4a3 and Col4a4 in a mouse model of Alport syndrome
-
Lu W, Phillips CL, Killen PD, Hlaing T, Harrison WR, Elder FF, Miner JH, Overbeek PA, Meisler MH: Insertional mutation of the collagen genes Col4a3 and Col4a4 in a mouse model of Alport syndrome. Genomics 61:113-124 (1999b).
-
(1999)
Genomics
, vol.61
, pp. 113-124
-
-
Lu, W.1
Phillips, C.L.2
Killen, P.D.3
Hlaing, T.4
Harrison, W.R.5
Elder, F.F.6
Miner, J.H.7
Overbeek, P.A.8
Meisler, M.H.9
-
34
-
-
0038146960
-
Immunopathology and the gene therapy of lupus
-
Mageed RA, Prud'homme GJ: Immunopathology and the gene therapy of lupus. Gene Therapy 10:861-874 (2003).
-
(2003)
Gene Therapy
, vol.10
, pp. 861-874
-
-
Mageed, R.A.1
Prud'homme, G.J.2
-
35
-
-
0030457613
-
Molecular and functional defects in kidneys of mice lacking alphacollagen3(IV): Implications for Alport syndrome
-
Miner JH, Sanes JR: Molecular and functional defects in kidneys of mice lacking alphacollagen3(IV): Implications for Alport syndrome. J Cell Biol 135:1403-1413 (1996).
-
(1996)
J Cell Biol
, vol.135
, pp. 1403-1413
-
-
Miner, J.H.1
Sanes, J.R.2
-
36
-
-
0028168648
-
Identification of mutations in the α3(IV) and α4(IV) collagen genes in autosomal recessive Alport syndrome
-
Mochizuki T, Lemmink HH, Mariyama M, Antignac C, Gubler MC, Pirson Y, Verellen-Dumoulin C, Chan B, Schroder CH, Smeets HJ, Reeders ST: Identification of mutations in the α3(IV) and α4(IV) collagen genes in autosomal recessive Alport syndrome. Nat Genet 8:77-82 (1994).
-
(1994)
Nat Genet
, vol.8
, pp. 77-82
-
-
Mochizuki, T.1
Lemmink, H.H.2
Mariyama, M.3
Antignac, C.4
Gubler, M.C.5
Pirson, Y.6
Verellen-Dumoulin, C.7
Chan, B.8
Schroder, C.H.9
Smeets, H.J.10
Reeders, S.T.11
-
37
-
-
0034815595
-
Murine lupus genetics: Lessons learned
-
Mohan C: Murine lupus genetics: Lessons learned. Curr Opin Rheumatol 13:352-360 (2001).
-
(2001)
Curr Opin Rheumatol
, vol.13
, pp. 352-360
-
-
Mohan, C.1
-
38
-
-
0032402990
-
Susceptibility to lupus nephritis in the NZB/W model system
-
Morel L, Wakeland ED: Susceptibility to lupus nephritis in the NZB/W model system. Curr Opin Immunol 10:718-725 (1998).
-
(1998)
Curr Opin Immunol
, vol.10
, pp. 718-725
-
-
Morel, L.1
Wakeland, E.D.2
-
39
-
-
34247574562
-
Susceptibility genes in the pathogenesis of murine lupus
-
Nguyen C, Limaye N, Wakeland EK: Susceptibility genes in the pathogenesis of murine lupus. Arthritis Res 4:S255-S263 (2002).
-
(2002)
Arthritis Res
, vol.4
-
-
Nguyen, C.1
Limaye, N.2
Wakeland, E.K.3
-
40
-
-
0029559014
-
Structural organization and expression of the mouse gene encoding alpha-galactosidase A
-
Ohshima T, Murray GJ, Nagle JW, Quirk JM, Kraus MH, Barton NW, Brady RO, Kulkarni AB: Structural organization and expression of the mouse gene encoding alpha-galactosidase A. Gene 166:277-280 (1995).
-
(1995)
Gene
, vol.166
, pp. 277-280
-
-
Ohshima, T.1
Murray, G.J.2
Nagle, J.W.3
Quirk, J.M.4
Kraus, M.H.5
Barton, N.W.6
Brady, R.O.7
Kulkarni, A.B.8
-
41
-
-
12644284502
-
α-Galactosidase A deficient mice: A model of Fabry disease
-
Ohshima T, Murray GJ, Swaim WD, Longenecker G, Quirk JM, Cardarelli CO, Sugimoto Y, Pastan I, Gottesman MM, Brady RO, Kulkarni AB: α-Galactosidase A deficient mice: A model of Fabry disease. Proc Natl Acad Sci USA 94:2540-2544 (1997).
-
(1997)
Proc Natl Acad Sci USA
, vol.94
, pp. 2540-2544
-
-
Ohshima, T.1
Murray, G.J.2
Swaim, W.D.3
Longenecker, G.4
Quirk, J.M.5
Cardarelli, C.O.6
Sugimoto, Y.7
Pastan, I.8
Gottesman, M.M.9
Brady, R.O.10
Kulkarni, A.B.11
-
42
-
-
13044281563
-
Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice
-
Ohshima T, Schiffmann R, Murray GJ, Kopp J, Quirk JM, Stahl S, Chan C-C, Zerfas P, Tao-Cheng J-H, Ward JM, Brady RO, Kulkarni AB: Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. Proc Natl Acad Sci USA 96:6423-6427 (1999).
-
(1999)
Proc Natl Acad Sci USA
, vol.96
, pp. 6423-6427
-
-
Ohshima, T.1
Schiffmann, R.2
Murray, G.J.3
Kopp, J.4
Quirk, J.M.5
Stahl, S.6
Chan, C.-C.7
Zerfas, P.8
Tao-Cheng, J.-H.9
Ward, J.M.10
Brady, R.O.11
Kulkarni, A.B.12
-
43
-
-
0028909692
-
Epidermal growth factor receptor expression is abnormal in murine polycystic kidney
-
Orellana SA, Sweeney WE, Neff CD, Avner ED: Epidermal growth factor receptor expression is abnormal in murine polycystic kidney. Kidney Int 47:490-499 (1995).
-
(1995)
Kidney Int
, vol.47
, pp. 490-499
-
-
Orellana, S.A.1
Sweeney, W.E.2
Neff, C.D.3
Avner, E.D.4
-
44
-
-
0037453003
-
Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transfer
-
Park J, Murray GJ, Limaye A, Quirk JM, Gelderman MP, Brady RO, Qasba P: Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transfer. Proc Natl Acad Sci USA 100:3450-3454 (2003).
-
(2003)
Proc Natl Acad Sci USA
, vol.100
, pp. 3450-3454
-
-
Park, J.1
Murray, G.J.2
Limaye, A.3
Quirk, J.M.4
Gelderman, M.P.5
Brady, R.O.6
Qasba, P.7
-
45
-
-
0033033706
-
Somatic PKD2 mutations in individual kidney and liver cysts support a "two-hit" model of cystogenesis in type 2 autosomal dominant polycystic kidney disease
-
Pei Y, Watnick T, He N, Wang K, Liang Y, Parfrey, P, Germino G, St George-Hyslop P: Somatic PKD2 mutations in individual kidney and liver cysts support a "two-hit" model of cystogenesis in type 2 autosomal dominant polycystic kidney disease. J Am Soc Nephrol 10:1524-1529 (1999).
-
(1999)
J Am Soc Nephrol
, vol.10
, pp. 1524-1529
-
-
Pei, Y.1
Watnick, T.2
He, N.3
Wang, K.4
Liang, Y.5
Parfrey, P.6
Germino, G.7
St George-Hyslop, P.8
-
46
-
-
0035960436
-
Autosomal dominant polycystic kidney disease: Modification of disease progression
-
Peters DJM, Breuning MH: Autosomal dominant polycystic kidney disease: modification of disease progression. Lancet 358:1439-1444 (2001).
-
(2001)
Lancet
, vol.358
, pp. 1439-1444
-
-
Peters, D.J.M.1
Breuning, M.H.2
-
47
-
-
0034111184
-
Enalapril in paediatric patients with Alport syndrome: Two years' experience
-
Proesmans W, Knockaert H, Trouet D: Enalapril in paediatric patients with Alport syndrome: two years' experience. Eur J Pediatr 159:430-433 (2000).
-
(2000)
Eur J Pediatr
, vol.159
, pp. 430-433
-
-
Proesmans, W.1
Knockaert, H.2
Trouet, D.3
-
48
-
-
0030582668
-
The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I
-
Qian F, Watnick TJ, Onuchic LF, Germino GG: The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell 87:979-987 (1996).
-
(1996)
Cell
, vol.87
, pp. 979-987
-
-
Qian, F.1
Watnick, T.J.2
Onuchic, L.F.3
Germino, G.G.4
-
49
-
-
0034999513
-
Treatment prospects for autosomal-dominant polycystic kidney disease
-
Qian Q, Harris PC, Torres VE: Treatment prospects for autosomal-dominant polycystic kidney disease. Kidney Int 59:2005-2022 (2001).
-
(2001)
Kidney Int
, vol.59
, pp. 2005-2022
-
-
Qian, Q.1
Harris, P.C.2
Torres, V.E.3
-
50
-
-
0035853120
-
Preselective gene therapy for Fabry disease
-
Qin G, Takenaka T, Telsch K, Kelley L, Howard T, Levade T, Deans R, Howard BH, Malech HL, Brady RO, Medin JA: Preselective gene therapy for Fabry disease. Proc Natl Acad Sci USA 98:3428-3433 (2001).
-
(2001)
Proc Natl Acad Sci USA
, vol.98
, pp. 3428-3433
-
-
Qin, G.1
Takenaka, T.2
Telsch, K.3
Kelley, L.4
Howard, T.5
Levade, T.6
Deans, R.7
Howard, B.H.8
Malech, H.L.9
Brady, R.O.10
Medin, J.A.11
-
51
-
-
0029021257
-
Modulation of disease activity in murine systemic lupus erythematosus by cytokine gene delivery
-
Raz E, Dudler J, Lotz M, Baird SM, Berry CC, Eisenberg RA, Carson DA: Modulation of disease activity in murine systemic lupus erythematosus by cytokine gene delivery. Lupus 4:286-292 (1995).
-
(1995)
Lupus
, vol.4
, pp. 286-292
-
-
Raz, E.1
Dudler, J.2
Lotz, M.3
Baird, S.M.4
Berry, C.C.5
Eisenberg, R.A.6
Carson, D.A.7
-
52
-
-
0026894270
-
Multilocus polycystic disease
-
Reeders ST: Multilocus polycystic disease. Nat Genet 1:235-237 (1992).
-
(1992)
Nat Genet
, vol.1
, pp. 235-237
-
-
Reeders, S.T.1
-
53
-
-
12944265457
-
Infusion of alpha-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease
-
Schiffmann R, Murray GJ, Treco D, Daniel P, Sellos-Moura M, Myers M, Quirk JM, Zirzow GC, Borowski M, Loveday K, Anderson T, Gillespie F, Oliver KL, Jeffries NO, Doo E, Liang TJ, Kreps C, Gunter K, Frei K, Crutchfield K, Selden RF, Brady RO: Infusion of alpha-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease. Proc Natl Acad Sci USA 97:364-370 (2000).
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 364-370
-
-
Schiffmann, R.1
Murray, G.J.2
Treco, D.3
Daniel, P.4
Sellos-Moura, M.5
Myers, M.6
Quirk, J.M.7
Zirzow, G.C.8
Borowski, M.9
Loveday, K.10
Anderson, T.11
Gillespie, F.12
Oliver, K.L.13
Jeffries, N.O.14
Doo, E.15
Liang, T.J.16
Kreps, C.17
Gunter, K.18
Frei, K.19
Crutchfield, K.20
Selden, R.F.21
Brady, R.O.22
more..
-
54
-
-
0035816007
-
Enzyme replacement therapy in Fabry disease: A randomized controlled trial
-
Schiffmann R, Koop JB, Austin HA, Sabnis S, Moore DF, Weibel T, Balow JE, Brady RO: Enzyme replacement therapy in Fabry disease: A randomized controlled trial. JAMA 285:2743-2776 (2001).
-
(2001)
JAMA
, vol.285
, pp. 2743-2776
-
-
Schiffmann, R.1
Koop, J.B.2
Austin, H.A.3
Sabnis, S.4
Moore, D.F.5
Weibel, T.6
Balow, J.E.7
Brady, R.O.8
-
55
-
-
0041519228
-
Renal involvement in Anderson-Fabry disease
-
Sessa A, Meroni M, Battini G, Righetti M, Maglio A, Tosoni A, Nebuloni M, Vago G, Giordano F: Renal involvement in Anderson-Fabry disease. J Nephrol 16:310-313 (2003).
-
(2003)
J Nephrol
, vol.16
, pp. 310-313
-
-
Sessa, A.1
Meroni, M.2
Battini, G.3
Righetti, M.4
Maglio, A.5
Tosoni, A.6
Nebuloni, M.7
Vago, G.8
Giordano, F.9
-
56
-
-
0037320210
-
Autosomal dominant polycystic kidney disease: Molecular genetics and pathophysiology
-
Sutters M, Germino GG: Autosomal dominant polycystic kidney disease: Molecular genetics and pathophysiology. J Lab Clin Med 141:91-101 (2003).
-
(2003)
J Lab Clin Med
, vol.141
, pp. 91-101
-
-
Sutters, M.1
Germino, G.G.2
-
57
-
-
0033995015
-
Treatment of polycystic kidney disease with a novel tyrosine kinase inhibitor
-
Sweeney WE, Chen Y, Nakanishi K, Frost P, Avner ED: Treatment of polycystic kidney disease with a novel tyrosine kinase inhibitor. Kidney Int 57:33-40 (2000).
-
(2000)
Kidney Int
, vol.57
, pp. 33-40
-
-
Sweeney, W.E.1
Chen, Y.2
Nakanishi, K.3
Frost, P.4
Avner, E.D.5
-
58
-
-
0141451943
-
Combination treatment of PKD utilizing dual inhibition of EGF-receptor activity and ligand bioavailability
-
Sweeney WE, Hamahira K, Sweeney J, Garcia-Gatrell M, Frost P, Avner ED: Combination treatment of PKD utilizing dual inhibition of EGF-receptor activity and ligand bioavailability. Kidney Int 64:1310-1319 (2003).
-
(2003)
Kidney Int
, vol.64
, pp. 1310-1319
-
-
Sweeney, W.E.1
Hamahira, K.2
Sweeney, J.3
Garcia-Gatrell, M.4
Frost, P.5
Avner, E.D.6
-
59
-
-
0037109052
-
Long-term systemic therapy of Fabry disease in a knockout mouse by adeno-associated virus-mediated muscle-directed gene transfer
-
Takahashi H, Hirai Y, Migita M, Seino Y, Fukuda Y, Sakuraba H, Kase R, Kobayashi T, Hashimoto Y, Shimada T: Long-term systemic therapy of Fabry disease in a knockout mouse by adeno-associated virus-mediated muscle-directed gene transfer. Proc Natl Acad Sci USA 99:13777-13782 (2002).
-
(2002)
Proc Natl Acad Sci USA
, vol.99
, pp. 13777-13782
-
-
Takahashi, H.1
Hirai, Y.2
Migita, M.3
Seino, Y.4
Fukuda, Y.5
Sakuraba, H.6
Kase, R.7
Kobayashi, T.8
Hashimoto, Y.9
Shimada, T.10
-
60
-
-
0033976034
-
CTLA4 IgG gene delivery prevents autoantibody production and lupus nephritis in MRL/lpr mice
-
Takiguchi M, Murakami M, Nakagawa I, Saito I, Hashimoto A, Uede T: CTLA4 IgG gene delivery prevents autoantibody production and lupus nephritis in MRL/lpr mice. Life Sci 66:991-1001 (2000).
-
(2000)
Life Sci
, vol.66
, pp. 991-1001
-
-
Takiguchi, M.1
Murakami, M.2
Nakagawa, I.3
Saito, I.4
Hashimoto, A.5
Uede, T.6
-
61
-
-
0022273898
-
Murine models of lupus systemic erythematosus
-
Theophiloulos AN, Dixon FJ: Murine models of lupus systemic erythematosus. Adv Immunol 37:269-290 (1985).
-
(1985)
Adv Immunol
, vol.37
, pp. 269-290
-
-
Theophiloulos, A.N.1
Dixon, F.J.2
-
62
-
-
0031798859
-
Upregulation of renal and systemic cyclooxygenase-2 in patients with active lupus nephritis
-
Tomasoni S, Noris M, Zappella S, Gotti E, Casiraghi F, Bonazzola S, Benigni A, Remuzzi G: Upregulation of renal and systemic cyclooxygenase-2 in patients with active lupus nephritis. J Am Soc Nephrol 9:1202-1212 (1998).
-
(1998)
J Am Soc Nephrol
, vol.9
, pp. 1202-1212
-
-
Tomasoni, S.1
Noris, M.2
Zappella, S.3
Gotti, E.4
Casiraghi, F.5
Bonazzola, S.6
Benigni, A.7
Remuzzi, G.8
-
63
-
-
0033153428
-
Genetic modifiers of polycystic kidney disease in intersubspecific KAT2J mutants
-
Upadhya P, Churchill G, Birkenmeier E, Barker J, Frankel W: Genetic modifiers of polycystic kidney disease in intersubspecific KAT2J mutants. Genomics 58:129-137 (1999).
-
(1999)
Genomics
, vol.58
, pp. 129-137
-
-
Upadhya, P.1
Churchill, G.2
Birkenmeier, E.3
Barker, J.4
Frankel, W.5
-
65
-
-
0034816964
-
Delineating the genetic basis of systemic lupus erythematosus
-
Wakeland EK, Liu K, Graham RR, Behrens TW: Delineating the genetic basis of systemic lupus erythematosus. Immunity 15:397-408 (2001).
-
(2001)
Immunity
, vol.15
, pp. 397-408
-
-
Wakeland, E.K.1
Liu, K.2
Graham, R.R.3
Behrens, T.W.4
-
66
-
-
0006885985
-
Progression of polycystic kidney disease in cpk mice is a quantitative trait under polygenic control
-
Woo D, Miao S, Tran T: Progression of polycystic kidney disease in cpk mice is a quantitative trait under polygenic control (Abstract). J Am Soc Nephrol 6:731A (1995).
-
(1995)
J Am Soc Nephrol
, vol.6
-
-
Woo, D.1
Miao, S.2
Tran, T.3
-
67
-
-
0030776906
-
Genetic identification of two major modifier loci of polycystic kidney disease progression in pcy mice
-
Woo D, Nguyen D, Khatibi N, Olsen P: Genetic identification of two major modifier loci of polycystic kidney disease progression in pcy mice. J Clin Invest 100:1934-1940 (1997).
-
(1997)
J Clin Invest
, vol.100
, pp. 1934-1940
-
-
Woo, D.1
Nguyen, D.2
Khatibi, N.3
Olsen, P.4
-
68
-
-
0032540226
-
Somatic inactivation of Pkd2 results in polycystic kidney disease
-
Wu G, D'Agati V, Cai Y, Markowitz G, Hoon Park J, Reynolds MD, Maeda Y, Le TC, Hou H, Kucherlapati R, Edelmann W, Somlo S: Somatic inactivation of Pkd2 results in polycystic kidney disease. Cell 93:177-188 (1998).
-
(1998)
Cell
, vol.93
, pp. 177-188
-
-
Wu, G.1
D'Agati, V.2
Cai, Y.3
Markowitz, G.4
Hoon Park, J.5
Reynolds, M.D.6
Maeda, Y.7
Le, T.C.8
Hou, H.9
Kucherlapati, R.10
Edelmann, W.11
Somlo, S.12
-
69
-
-
0030776593
-
Renal expression of monocyte chemoattractant protein-1 in lupus autoimmune mice
-
Zoja C, Liu X-H, Donadelli R, Abbate M, Testa D, Corna D, Taraboletti G, Vecchi A, Dong QG, Rollins BJ, Bertani T, Remuzzi G: Renal expression of monocyte chemoattractant protein-1 in lupus autoimmune mice. J Am Soc Nephrol 8:720-729 (1997).
-
(1997)
J Am Soc Nephrol
, vol.8
, pp. 720-729
-
-
Zoja, C.1
Liu, X.-H.2
Donadelli, R.3
Abbate, M.4
Testa, D.5
Corna, D.6
Taraboletti, G.7
Vecchi, A.8
Dong, Q.G.9
Rollins, B.J.10
Bertani, T.11
Remuzzi, G.12
-
70
-
-
0031940491
-
Bindarit retards renal disease and prolongs survival in murine lupus autoimmune disease
-
Zoja C, Corna D, Benedetti G, Morigi M, Donadelli R, Guglielmotti A, Pinza M, Bertani T, Remuzzi G: Bindarit retards renal disease and prolongs survival in murine lupus autoimmune disease. Kidney Int 53:726-734 (1998).
-
(1998)
Kidney Int
, vol.53
, pp. 726-734
-
-
Zoja, C.1
Corna, D.2
Benedetti, G.3
Morigi, M.4
Donadelli, R.5
Guglielmotti, A.6
Pinza, M.7
Bertani, T.8
Remuzzi, G.9
-
71
-
-
0034927121
-
Mycophenolate mofetil combined with a cyclooxygenase-2 inhibitor ameliorates murine lupus nephritis
-
Zoja C, Benigni A, Noris M, Corna D, Casiraghi F, Pagnoncelli M, Rottoli D, Abbate M, Remuzzi G: Mycophenolate mofetil combined with a cyclooxygenase-2 inhibitor ameliorates murine lupus nephritis. Kidney Int 60:653-663 (2001).
-
(2001)
Kidney Int
, vol.60
, pp. 653-663
-
-
Zoja, C.1
Benigni, A.2
Noris, M.3
Corna, D.4
Casiraghi, F.5
Pagnoncelli, M.6
Rottoli, D.7
Abbate, M.8
Remuzzi, G.9
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