-
2
-
-
0023150727
-
Cysteamine therapy for children with nephropathic cystinosis
-
Gahl W.A., Reed G.F., Thoene J.G. et al. Cysteamine therapy for children with nephropathic cystinosis. N. Engl. J. Med. (1987) 316 971-977.
-
(1987)
N. Engl. J. Med.
, vol.316
, pp. 971-977
-
-
Gahl, W.A.1
Reed, G.F.2
Thoene, J.G.3
-
3
-
-
0027478064
-
Improved renal function in children with cystinosis treated with cysteamine
-
Markello T.C., Bernardini I.M., Gahl W.A. Improved renal function in children with cystinosis treated with cysteamine. N. Engl. J. Med. (1993) 328 1157-1162.
-
(1993)
N. Engl. J. Med.
, vol.328
, pp. 1157-1162
-
-
Markello, T.C.1
Bernardini, I.M.2
Gahl, W.A.3
-
4
-
-
5144225376
-
Long-term follow-up of well-treated nephropathic cystinosis patients
-
Kleta R., Bernardini I., Ueda M. et al. Long-term follow-up of well-treated nephropathic cystinosis patients. J. Pediatr. (2004) 145 555-560.
-
(2004)
J. Pediatr.
, vol.145
, pp. 555-560
-
-
Kleta, R.1
Bernardini, I.2
Ueda, M.3
-
5
-
-
79953723142
-
Enteric-coated cysteamine for the treatment of paediatric non-alcoholic fatty liver disease
-
Dohil R., Schmeltzer S., Cabrera B.L. et al. Enteric-coated cysteamine for the treatment of paediatric non-alcoholic fatty liver disease. Aliment. Pharmacol. Ther. (2011) 33 1036-1044.
-
(2011)
Aliment. Pharmacol. Ther.
, vol.33
, pp. 1036-1044
-
-
Dohil, R.1
Schmeltzer, S.2
Cabrera, B.L.3
-
6
-
-
0037109665
-
Therapeutic effects of cystamine in a murine model of Huntington's disease
-
Dedeoglu A., Kubilus J.K., Jeitner T.M. et al. Therapeutic effects of cystamine in a murine model of Huntington's disease. J. Neurosci. (2002) 22 8942-8950.
-
(2002)
J. Neurosci.
, vol.22
, pp. 8942-8950
-
-
Dedeoglu, A.1
Kubilus, J.K.2
Jeitner, T.M.3
-
7
-
-
0037423204
-
Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders
-
Lesort M., Lee M., Tucholski J., Johnson G.V. Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders. J. Biol. Chem. (2003) 278 3825-3830.
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 3825-3830
-
-
Lesort, M.1
Lee, M.2
Tucholski, J.3
Johnson, G.V.4
-
8
-
-
41049099937
-
Antioxidant effect of cysteamine in brain cortex of young rats
-
Kessler A., Biasibetti M., da Silva Melo D.A. et al. Antioxidant effect of cysteamine in brain cortex of young rats. Neurochem. Res. (2008) 33 737-744.
-
(2008)
Neurochem. Res.
, vol.33
, pp. 737-744
-
-
Kessler, A.1
Biasibetti, M.2
da Silva Melo, D.A.3
-
9
-
-
39149104655
-
Effects of cysteamine on oxidative status in cerebral cortex of rats
-
Kessler A., Biasibetti M., Feksa L.R. et al. Effects of cysteamine on oxidative status in cerebral cortex of rats. Metab. Brain Dis. (2008) 23 81-93.
-
(2008)
Metab. Brain Dis.
, vol.23
, pp. 81-93
-
-
Kessler, A.1
Biasibetti, M.2
Feksa, L.R.3
-
10
-
-
53749089841
-
A novel approach to enhancing cellular glutathione levels
-
Maher P., Lewerenz J., Lozano C., Torres J.L. A novel approach to enhancing cellular glutathione levels. J. Neurochem. (2008) 107 690-700.
-
(2008)
J. Neurochem.
, vol.107
, pp. 690-700
-
-
Maher, P.1
Lewerenz, J.2
Lozano, C.3
Torres, J.L.4
-
11
-
-
34447558473
-
Cellular thiol pools are responsible for sequestration of cytotoxic reactive aldehydes: central role of free cysteine and cysteamine
-
Wood P.L., Khan M.A., Moskal J.R. Cellular thiol pools are responsible for sequestration of cytotoxic reactive aldehydes: central role of free cysteine and cysteamine. Brain Res. (2007) 1158 158-163.
-
(2007)
Brain Res.
, vol.1158
, pp. 158-163
-
-
Wood, P.L.1
Khan, M.A.2
Moskal, J.R.3
-
12
-
-
0031764609
-
DRPLA aggregation and transglutaminase, revisited
-
Lorand L. DRPLA aggregation and transglutaminase, revisited. Nat. Genet. (1998) 20 231.
-
(1998)
Nat. Genet.
, vol.20
, pp. 231
-
-
Lorand, L.1
-
13
-
-
14644415488
-
Mechanism for the inhibition of transglutaminase 2 by cystamine
-
Jeitner T.M., Delikatny E.J., Ahlqvist J., Capper H., Cooper A.J. Mechanism for the inhibition of transglutaminase 2 by cystamine. Biochem. Pharmacol. (2005) 69 961-970.
-
(2005)
Biochem. Pharmacol.
, vol.69
, pp. 961-970
-
-
Jeitner, T.M.1
Delikatny, E.J.2
Ahlqvist, J.3
Capper, H.4
Cooper, A.J.5
-
14
-
-
77949889225
-
Transglutaminase-catalyzed crosslinking in neurological disease: from experimental evidence to therapeutic inhibition
-
Hoffner G., Andre W., Vanhoutteghem A., Soues S., Djian P. Transglutaminase-catalyzed crosslinking in neurological disease: from experimental evidence to therapeutic inhibition. CNS Neurol. Disord. Drug. Targets (2010) 9 217-231.
-
(2010)
CNS Neurol. Disord. Drug. Targets
, vol.9
, pp. 217-231
-
-
Hoffner, G.1
Andre, W.2
Vanhoutteghem, A.3
Soues, S.4
Djian, P.5
-
15
-
-
33744931367
-
Understanding intestinal cysteamine bitartrate absorption
-
Dohil R., Fidler M., Barshop B.A. et al. Understanding intestinal cysteamine bitartrate absorption. J. Pediatr. (2006) 148 764-769.
-
(2006)
J. Pediatr.
, vol.148
, pp. 764-769
-
-
Dohil, R.1
Fidler, M.2
Barshop, B.A.3
-
16
-
-
72049095333
-
Twice-daily cysteamine bitartrate therapy for children with cystinosis
-
Dohil R., Fidler M., Gangoiti J.A., Kaskel F., Schneider J.A., Barshop B.A. Twice-daily cysteamine bitartrate therapy for children with cystinosis. J. Pediatr. (2010) 156 71-75.
-
(2010)
J. Pediatr.
, vol.156
, pp. 71-75
-
-
Dohil, R.1
Fidler, M.2
Gangoiti, J.A.3
Kaskel, F.4
Schneider, J.A.5
Barshop, B.A.6
-
17
-
-
77950520525
-
Long-term treatment of cystinosis in children with twice-daily cysteamine
-
Dohil R., Gangoiti J.A., Cabrera B.L., Fidler M., Schneider J.A., Barshop B.A. Long-term treatment of cystinosis in children with twice-daily cysteamine. J. Pediatr. (2010) 156 823-827.
-
(2010)
J. Pediatr.
, vol.156
, pp. 823-827
-
-
Dohil, R.1
Gangoiti, J.A.2
Cabrera, B.L.3
Fidler, M.4
Schneider, J.A.5
Barshop, B.A.6
-
18
-
-
84876281217
-
A randomized controlled crossover trial with RP103 for nephropathic cystinosis
-
Langman C.B., Greenbaum L.A., Sarwar M. et al. A randomized controlled crossover trial with RP103 for nephropathic cystinosis. Clin. J. Am. Soc. Nephrol. (2012) 7 112-120
-
(2012)
Clin. J. Am. Soc. Nephrol.
, vol.7
, pp. 112-120
-
-
Langman, C.B.1
Greenbaum, L.A.2
Sarwar, M.3
-
19
-
-
0017688457
-
Gastric emptying in the rat is inhibited by the duodenal ulcerogens, cysteamine and propionitrile
-
Lichtenberger L.M., Szabo S., Reynolds E.S. Gastric emptying in the rat is inhibited by the duodenal ulcerogens, cysteamine and propionitrile. Gastroenterology (1977) 73 1072-1076.
-
(1977)
Gastroenterology
, vol.73
, pp. 1072-1076
-
-
Lichtenberger, L.M.1
Szabo, S.2
Reynolds, E.S.3
-
20
-
-
33845410994
-
Pharmacokinetics of cysteamine bitartrate following gastrointestinal infusion
-
Fidler M.C., Barshop B.A., Gangoiti J.A. et al. Pharmacokinetics of cysteamine bitartrate following gastrointestinal infusion. Br. J. Clin. Pharmacol. (2007) 63 36-40.
-
(2007)
Br. J. Clin. Pharmacol.
, vol.63
, pp. 36-40
-
-
Fidler, M.C.1
Barshop, B.A.2
Gangoiti, J.A.3
-
21
-
-
0018028416
-
Duodenal ulcer disease. Animal model: cysteamine-induced acute and chronic duodenal ulcer in the rat
-
Szabo S. Duodenal ulcer disease. Animal model: cysteamine-induced acute and chronic duodenal ulcer in the rat. Am. J. Pathol. (1978) 93 273-276.
-
(1978)
Am. J. Pathol.
, vol.93
, pp. 273-276
-
-
Szabo, S.1
-
22
-
-
0242575995
-
Steady-state pharmacokinetics and pharmacodynamics of cysteamine bitartrate in paediatric nephropathic cystinosis patients
-
Belldina E.B., Huang M.Y., Schneider J.A., Brundage R.C., Tracy T.S. Steady-state pharmacokinetics and pharmacodynamics of cysteamine bitartrate in paediatric nephropathic cystinosis patients. Br. J. Clin. Pharmacol. (2003) 56 520-525.
-
(2003)
Br. J. Clin. Pharmacol.
, vol.56
, pp. 520-525
-
-
Belldina, E.B.1
Huang, M.Y.2
Schneider, J.A.3
Brundage, R.C.4
Tracy, T.S.5
-
23
-
-
77049187634
-
Cysteamine-cystamine: intestinal absorption, distribution among various organs and excretion
-
Eldjarn L., Nygaard O. Cysteamine-cystamine: intestinal absorption, distribution among various organs and excretion. Arch. Int. Physiol. Biochim. (1954) 62 476-486.
-
(1954)
Arch. Int. Physiol. Biochim.
, vol.62
, pp. 476-486
-
-
Eldjarn, L.1
Nygaard, O.2
-
24
-
-
18744364173
-
Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy
-
Sonies B.C., Almajid P., Kleta R., Bernardini I., Gahl W.A. Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy. Medicine (Baltimore) (2005) 84 137-146.
-
(2005)
Medicine (Baltimore)
, vol.84
, pp. 137-146
-
-
Sonies, B.C.1
Almajid, P.2
Kleta, R.3
Bernardini, I.4
Gahl, W.A.5
-
25
-
-
84864191003
-
New mechanistic explanation for the localization of ulcers in the rat duodenum: role of iron and selective uptake of cysteamine
-
Khomenko T., Kolodney J., Pinto J.T. et al. New mechanistic explanation for the localization of ulcers in the rat duodenum: role of iron and selective uptake of cysteamine. Arch. Biochem. Biophys. (2012) 55 60-70
-
(2012)
Arch. Biochem. Biophys.
, vol.55
, pp. 60-70
-
-
Khomenko, T.1
Kolodney, J.2
Pinto, J.T.3
-
26
-
-
0035989758
-
Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome
-
Levtchenko E.N., de Graaf-Hess A., Blom H.J., Monnens L.A. Negligible urinary cysteamine loss in cystinosis patients with Fanconi syndrome. Clin. Nephrol. (2002) 57 349-351.
-
(2002)
Clin. Nephrol.
, vol.57
, pp. 349-351
-
-
Levtchenko, E.N.1
de Graaf-Hess, A.2
Blom, H.J.3
Monnens, L.A.4
-
27
-
-
84865341244
-
A potential new method to estimate tissue cystine content in nephropathic cystinosis
-
Dohil R., Carrigg A., Newbury R. A potential new method to estimate tissue cystine content in nephropathic cystinosis. J. Pediatr. (2012) 161 531-535.
-
(2012)
J. Pediatr.
, vol.161
, pp. 531-535
-
-
Dohil, R.1
Carrigg, A.2
Newbury, R.3
-
28
-
-
0023156417
-
Removal of corneal crystals by topical cysteamine in nephropathic cystinosis
-
Kaiser-Kupfer M.I., Fujikawa L., Kuwabara T., Jain S., Gahl W.A. Removal of corneal crystals by topical cysteamine in nephropathic cystinosis. N. Engl. J. Med. (1987) 316 775-779.
-
(1987)
N. Engl. J. Med.
, vol.316
, pp. 775-779
-
-
Kaiser-Kupfer, M.I.1
Fujikawa, L.2
Kuwabara, T.3
Jain, S.4
Gahl, W.A.5
-
29
-
-
0036891796
-
Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells
-
Park M., Helip-Wooley A., Thoene J. Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells. J. Am. Soc. Nephrol. (2002) 13 2878-2887.
-
(2002)
J. Am. Soc. Nephrol.
, vol.13
, pp. 2878-2887
-
-
Park, M.1
Helip-Wooley, A.2
Thoene, J.3
-
30
-
-
0035084699
-
Nonalcoholic steatohepatitis: association of insulin resistance and mitochondrial abnormalities
-
Sanyal A.J., Campbell-Sargent C., Mirshahi F. et al. Nonalcoholic steatohepatitis: association of insulin resistance and mitochondrial abnormalities. Gastroenterology (2001) 120 1183-1192.
-
(2001)
Gastroenterology
, vol.120
, pp. 1183-1192
-
-
Sanyal, A.J.1
Campbell-Sargent, C.2
Mirshahi, F.3
-
31
-
-
77955722879
-
Pharmacokinetics of enteric-coated cysteamine bitartrate in healthy adults: a pilot study
-
Gangoiti J.A., Fidler M., Cabrera B.L., Schneider J.A., Barshop B.A., Dohil R. Pharmacokinetics of enteric-coated cysteamine bitartrate in healthy adults: a pilot study. Br. J. Clin. Pharmacol. (2010) 70 376-382.
-
(2010)
Br. J. Clin. Pharmacol.
, vol.70
, pp. 376-382
-
-
Gangoiti, J.A.1
Fidler, M.2
Cabrera, B.L.3
Schneider, J.A.4
Barshop, B.A.5
Dohil, R.6
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