Cysteamine prevents inhibition of thiol-containing enzymes caused by cystine or cystine dimethylester loading in rat brain cortex

Metabolic Brain Disease - Tập 23 Số 2 - Trang 133-145 - 2008
Virgínia Cielo Rech1, Luciane Rosa Feksa1, Rochele Marisa Müller Fleck1, Genaro Azambuja Athaydes1, Paula Karina Barcelos Dornelles1, Valnês S. Rodrigues-Junior1, Clóvis Milton Duval Wannmacher1
1Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, UFRGS, Porto Alegre, Brazil

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Andrews NW (2000) Regulated secretion of conventional lysosomes. Trends Cell Biol 10:316–321

Armstrong JS, Jones DP (2002) Glutathione depletion enforces the mitochondrial permeability transition and causes cell death in Bcl-2 overexpressing HL60 cells. FASEB J 16:1263–1265

Baum M (1998) The Fanconi syndrome of cystinosis: insights into the pathophysiology. Pediatr Nephrol 12:492–497

Ben-Nun A, Bashan N, Potashnik R, Cohen-Luria R, Moran A (1993) Cystine loading induces Fanconi’s syndrome in rats: in vivo and vesicle studies. Am J Physiol 265:839–844

Bergeron M, Gougoux A, Noël J, Parent L (2001) The renal Fanconi syndrome. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic & molecular bases of inherited diseases, 8th edn. McGraw-Hill, New York, pp 5023–5083

Berry EV, Toms NJ (2006) Pyruvate and oxaloacetate limit zinc-induced oxidative HT-22 neuronal cell injury. Neurotoxicol 27:1043–1051

Burlacu A, Jinga V, Gafencu AV, Simionescu M (2001) Severity of oxidative stress generates different mechanisms of endothelial cell death. Cell Tissue Res 306:409–416

Çcetinkaya I, Schlatter E, Hirsch JR, Herter P, Harms E, Kleta R (2002) Inhibition of Na+-dependent transporters in cystine-loaded human renal cells: electrophysiological studies on the Fanconi syndrome of cystinosis. J Am Soc Nephrol 13:2085–2093

Cherqui S, Sevin C, Hamard G, Kalatzis V, Sich M, Pequignot MO, Gogat K, Abitbol M, Broyer M, Gubler MC, Antignac C (2002) Intralysosomal cystine accumulation in mice lacking cystinosin, the protein defective in cystinosis. Mol Cell Biol 22:7622–7632

Chol M, Nevo N, Cherqui S, Antignac C, Rustin P (2004) Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines. Biochem Biophys Res Comm 324:231–235

Clarke PG (1990) Developmental cell death: morphological diversity and multiple mechanisms. Anat Embryol 181:195–213

Coor C, Salmon RF, Quigley R, Marver D, Baum M (1991) Role of adenosine- triphosphate (ATP) and Na+, K+-ATPase in the inhibition of proximal tubule transport with intracellular cystine loading. J Clin Invest 87:955–961

Das UN (2006) Pyruvate is an endogenous anti-inflammatory and anti-oxidant molecule. Med Sci Monit 12:RA79–RA84

Feksa LR, Cornelio AR, Dutra-Filho CS, Wyse AT, Wajner M, Wannmacher CMD (2004) Inhibition of pyruvate kinase activity by cystine in brain cortex of rats. Brain Res 1012:93–100

Fleck RM, Rodrigues Jr V, Giacomazzi J, Parissoto D, Dutra-Filho CS, Wyse AT, Wajner M, Wannmacher CMD (2005) Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex. Neurochem Int 46:391–397

Foreman JW, Benson L (1990) Effect of cystine loading and cystine dimethyl ester on renal brushborder membrane-transport. Biosci Rep 10:455–459

Foreman JW, Bowring MA, Lee J, States B, Segal S (1987) Effect of cystine dimethyl ester on renal solute handling and isolated renal tubule transport in the rat. A new model of the Fanconi syndrome. Metab Clin Exper 36:1185–1191

Foreman JW, Benson LL, Wellons M, Avner ED, Sweeney W, Nissim L, Nissim I (1995) Metabolic studies of rat renal tubule cells loaded with cystine: the cystine dimethyl ester model of cystinosis. J Am Soc Nephrol 6:269–272

Gahl WA (2003) Early oral cysteamine therapy for nephropathic cystinosis. Eur J Pediatr 162:S38–S41

Gahl WA, Charnas L, Markello TC, Bernardini I, Ishak KG, Dalakas MC (1992) Parenchymal organ cystine depletion with long-term cysteamine therapy. Biochem. Med Metab Biol 48:275–285

Gahl WA, Thoene JG, Schneider JA (2001) Cystinosis: a disorder of lysossomal membrane transport. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular bases of inherited disease, 8th edn. McGraw-Hill, New York, pp 5085–5108

Gahl WA, Thoene JG, Schneider JA (2002) Cystinosis. N Engl J Med 347:111–121

Gilbert HF (1984) Redox control of enzyme activities by thiol/disulfide exchange. Meth Enzymol 107:330–351

Hall ER, Cottam GL (1978) Isoenzymes of pyruvate kinase in vertebrates: their physical, chemical, kinetic and immunological properties. Int J Biochem 9:785–793

Hatano E, Tanaka A, Kanazawa A, Tsuyuki S, Tsunekawa S, Iwata S, Ellerby LM, Bredesen D, Freeze H, Abrahamson M, Bromme D, Krajewski S, Reed JC, Yin XM, Turk V (2004) Inhibition of tumor necrosis factor-induced apoptosis in transgenic mouse liver expressing creatine kinase. Liver Int 24:384–393

Hughes BP (1962) A method for the estimation of serum creatine kinase and its use in comparing creatine kinase and aldolase activity in normal and pathological sera. Clin Chim Acta 7:597–603

Jiang S, Moriarty-Craige SE, Orr M, Cai J, Sternberg P, Jones DP (2005) Oxidant-induced apoptosis in human retinal pigment epithelial cells: dependence on extracellular redox state. Invest Ophtalmol Vis Sci 46:1054–1061

Jonas AJ, Conley SB, Marshall R, Johnson RA, Marks M, Rosenberg H (1987) Nephropathic cystinosis with central nervous system involvement. Am J Med 83:966–970

Jones DP, Go YM, Anderson CL, Ziegler TR, Kinkade Jr JM, Kirlin WG (2004) Cysteine/cystine couple is a newly recognized node in the circuitry for biologic redox signaling and control. FASEB J 18:1246–1248

Kaplan A, Szabo LL, Opheim KE (1988) Clinical Chemistry: Interpretation and technique. Lea and Febiger, Philadelphia, pp 186–189

Laube GF, Shah V, Stewart VC, Hargreaves IP, Haq MR, Heales SJ, van’t Hoff WG (2006) Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells. Pediatr Nephrol 21:503–509

Leech NL, Barrett KC, Morgan GA (2005) SPSS for intermediate statistics. Use and interpretation, 2nd edn. Erlbaum, London

Leong SF, Lai JC, Lim L, Clark JB (1981) Energy-metabolising enzymes in brain regions of adult and aging rats. J Neurochem 37:1548–1556

Levtchenko E, Graaf-Hess A, Wilmer M, van der Heuvel L, Monnens L, Blom H (2005) Altered status of glutathione and its metabolites in cystinotic cells. Nephrol Dial Transplant 20:1828–1832

Levtchenko EN, Wilmer MJG, Janssen AJM, Koenderink JB, Visch AJ, Willems PH, Graaf-Hess A, Blom HJ, van den Heuvel LP, Monnens LA (2006) Decreased ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblasts. Pediatr Res 59:287–292

Lowry OH, Rosebrough NJ, Farr AL, Randall RJ (1951) Protein measurement with the Folin phenol reagent. J Biol Chem 193:265–275

Nichols SL, Press GA, Schneider JA, Trauner DA (1990) Cortical atrophy and cognitive performance in infantile nephropathic cystinosis. Pediatr Neurol 6:379–381

Park MA, Thoene JG (2005) Potential role of apoptosis in development of the cystinotic phenotype. Pediatr Nephrol 20:441–446

Park MA, Helip-Wooley A, Thoene J (2002) Lysosomal cystine storage increases apoptosis in cultured human fibroblasts and renal proximal tubule epithelial cells. J Am Soc Nephrol 13:2878–2887

Park MA, Pejovic V, Kerisit KG, Junius S, Thoene JG (2006) Increased apoptosis in cystinotic fibroblasts and renal proximal tubule epithelial cells results from cysteinylation of protein kinase C (delta). J Am Soc Nephrol 17:3167–3175

Patrick AD (1965) Deficiencies of SH-dependent enzymes in cystinosis. Clin Sci 28:427–443

Salmon RF, Baum M (1990) Intracellular cystine loading inhibits transport in the rabbit proximal convoluted tubule. J Clin Invest 85:340–344

Schneider JA, Schulman JD (1983) Cystinosis. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The metabolic basis of inherited disease, 5th edn. McGraw-Hill, New York, pp 1844–18566

Schneider JA, Clark KF, Greene AA, Reisch JS, Markello TC, Gahl WA, Thoene JG, Noonan PK, Berry KA (1983) Recent advances in the treatment of cystinosis. J Inher Metab Dis 18:387–397

Schulze A (2003) Creatine deficiency syndromes. Mol Cell Biochem 244:143–150

Sestili P, Martinelli C, Bravi G, Piccoli G, Curci R, Battistelli M, Falcieri E, Agostini D, Gioacchini AM, Stocchi V (2006) Creatine supplementation affords cytoprotection in oxidatively injured cultured mammalian cells via direct antioxidant activity. Free Radic Biol Med 40:837–849

Spear GS, Gubler MC, Habib R, Broyer M (1989) Dark cells of cystinosis: occurrence in renal allografts. Hum Pathol 20:472–476

Sullivan MX, Hess WC, Howard HW (1942) The quantitative estimation of both cystine and cystein in mixture. J Biol Chem 145:621–624

Town M, Jean G, Cherqui S, Attard M, Forestier L, Whitmore SA, Callen DF, Gribouval O, Broyer M, Bates GP, van’t Hoff W, Antignac C (1998) A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis. Nat Genet 18:319–324

Vogel DG, Malekzadeh MH, Cornford ME, Schneider JA, Shields WD, Vinters HV (1990) Central nervous system involvement in nephropathic cystinosis. J Neuropathol Exp Neurol 49:591–599

Wallimann T, Wyss M, Brdiczka D, Nicolay K, Eppenberger HM (1992) Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the ‘phosphocreatine circuit’ for cellular energy homeostasis. Biochem J 281:21–40

Wilmer MJ, de Graaf-Hess A, Blom HJ, Dijkman HB, Monnens LA, van den Heuvel LP, Levtchenko EN (2005) Elevated oxidized glutathione in cystinotic proximal tubular epithelial cells. Biochem Biophys Res Commun 337:610–614

Zahler WL, Cleland WW (1968) A specific and sensitive assay for disulfides. J Biol Chem 243:716–719