Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex

Fleck, Rochele Marisa Müller; Junior, Valnes Rodrigues; Giacomazzi, Juliana; Parissoto, Daiana; Dutra-Filho, Carlos Severo; Wyse, Ângela T. S.; Wajner, Moaçir; Wannmacher, Clóvis Milton Duval

doi:10.1016/j.neuint.2004.11.008

Cited by 16 publications

(9 citation statements)

References 49 publications

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“…We have already demonstrated that cystine inhibits in vitro CK activity in rat brain (13). In the present study, we demonstrate that cystine strongly inhibits in vivo and in vitro kidney CK activity and that cysteamine can prevent this inhibition.…”

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confidence: 77%

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Inhibition of Creatine Kinase Activity by Cystine in the Kidney of Young Rats

et al. 2006

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Nephropathic cystinosis is a lethal genetic disease caused by a lysosomal transport disorder leading to intralysosomal cystine accumulation in all tissues. Cystinosis is the most common inherited cause of Fanconi syndrome, but the mechanisms by which cystine causes tissue damage are not fully understood. Thiolcontaining enzymes are critical for renal energy metabolism and may be altered by disulfides like cystine. Therefore, in the present study our main objective was to investigate the in vivo and in vitro effects of cystine on creatine kinase, which contains critical thiol groups in its structure, in the kidney of young Wistar rats. We observed that cystine inhibited in vivo and in vitro the enzyme activity and that this inhibition was prevented by cysteamine and glutathione. The results suggest oxidation of essential sulfhydryl groups necessary for creatine kinase function by cystine. Considering that creatine kinase and other thiol-containing enzymes are crucial for renal energy metabolism, and programmed cell death occurs in situations of energy deficiency, the enzyme inhibition caused by cystine released from lysosomes might be a mechanism of tissue damage in patients with cystinosis. (Pediatr Res 60: 190-195, 2006)

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confidence: 77%

“…One of the factors that enable cells to undergo apoptosis, or other form of programmed cell death, is an alteration of mitochondrial permeability with consequent reduction of ATP supply (12). In this context, we have recently reported that cystine inhibits CK activity in vitro in the brain of young rats (13).…”

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Inhibition of Creatine Kinase Activity by Cystine in the Kidney of Young Rats

et al. 2006

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“…Without treatment, most children with cystinosis develop renal failure, visuospatial difficulties, and increasing discrepancy in a progressive cognitive impairment due to cystine accumulation in the brain [4,5]. Accumulated data indicated that at the molecule level, the high concentration of intracellular cystine affected the activity and functions of many thiol-containing enzymes, such as pyruvate kinase and creatine kinase [6][7][8][9].…”

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confidence: 99%

Generation of the oxidized form protects human brain type creatine kinase against cystine-induced inactivation

Chen

Gao

et al. 2011

International Journal of Biological Macromolecules

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“…In this context, creatine kinase inhibition has also been observed in neurodegenerative and mental diseases, such as Alzheimer's disease and schizophrenia [33]. Besides, it has been demonstrated that the enzyme is also inhibited in animal models of some inborn errors of metabolism affecting the brain [34][35][36][37]. Finally, it would be expected that reduced functional brain activity may result in cognitive deficits [38].…”

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confidence: 99%

Decreased Creatine Kinase Activity Caused by Electroconvulsive Shock

et al. 2006

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Although several advances have occurred over the past 20 years concerning the use and administration of electroconvulsive therapy to minimize side effects of this treatment, little progress has been made in understanding its mechanism of action. Creatine kinase is a crucial enzyme for brain energy homeostasis, and a decrease of its activity has been associated with neuronal death. This work was performed in order to evaluate creatine kinase activity from rat brain after acute and chronic electroconvulsive shock. Results showed an inhibition of creatine kinase activity in hippocampus, striatum and cortex, after acute and chronic electroconvulsive shock. Our findings demonstrated that creatine kinase activity is altered by electroconvulsive shock.

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Cysteamine prevents and reverses the inhibition of creatine kinase activity caused by cystine in rat brain cortex

Cited by 16 publications

References 49 publications

Inhibition of Creatine Kinase Activity by Cystine in the Kidney of Young Rats

Inhibition of Creatine Kinase Activity by Cystine in the Kidney of Young Rats

Generation of the oxidized form protects human brain type creatine kinase against cystine-induced inactivation

Decreased Creatine Kinase Activity Caused by Electroconvulsive Shock

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