Creatine transporter 1 (CT1) defect is an X-linked disease that causes severe neurological impairment. No treatment has been available for this condition so far. Because the transport of creatine (Cr) precursors Gly and Arg is not affected in this disorder, we tested the possible corrective effect of these two amino acids on Cr depletion in lymphoblasts lacking the transporter. Substrates enriched with Arg or Arg plus Gly increased the concentration of intracellular Cr in affected cells as well as in control cells. The greatest effect was obtained with 10 and 15 mM Arg and 10 mM Arg plus Gly. These results encourage an in vivo trial with Cr precursors in CT1 defect. (C) 2008 Elsevier Inc. All rights reserved.

Arginine and glycine stimulate creatine synthesis in creatine transporter 1-deficient lymphoblasts / Leuzzi, Vincenzo; Maria G., Alessandri; Manuela, Casarano; Roberta, Battini; Giovanni, Cioni. - In: ANALYTICAL BIOCHEMISTRY. - ISSN 0003-2697. - 375:1(2008), pp. 153-155. [10.1016/j.ab.2008.01.018]

Arginine and glycine stimulate creatine synthesis in creatine transporter 1-deficient lymphoblasts

LEUZZI, Vincenzo;
2008

Abstract

Creatine transporter 1 (CT1) defect is an X-linked disease that causes severe neurological impairment. No treatment has been available for this condition so far. Because the transport of creatine (Cr) precursors Gly and Arg is not affected in this disorder, we tested the possible corrective effect of these two amino acids on Cr depletion in lymphoblasts lacking the transporter. Substrates enriched with Arg or Arg plus Gly increased the concentration of intracellular Cr in affected cells as well as in control cells. The greatest effect was obtained with 10 and 15 mM Arg and 10 mM Arg plus Gly. These results encourage an in vivo trial with Cr precursors in CT1 defect. (C) 2008 Elsevier Inc. All rights reserved.
2008
01 Pubblicazione su rivista::01a Articolo in rivista
Arginine and glycine stimulate creatine synthesis in creatine transporter 1-deficient lymphoblasts / Leuzzi, Vincenzo; Maria G., Alessandri; Manuela, Casarano; Roberta, Battini; Giovanni, Cioni. - In: ANALYTICAL BIOCHEMISTRY. - ISSN 0003-2697. - 375:1(2008), pp. 153-155. [10.1016/j.ab.2008.01.018]
File allegati a questo prodotto
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/120562
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? 8
  • Scopus 17
  • ???jsp.display-item.citation.isi??? 16
social impact