Luiz Roberto Carvalho

Publications (2)8.69 Total impact

• Article: ACE activity is modulated by the enzyme α-galactosidase A.

  Elice Carneiro Batista, Luiz Roberto Carvalho, Dulce Elena Casarini, Adriana Karaoglanovic Carmona, Edson Lucas dos Santos, Elton Dias da Silva, Robson Augusto dos Santos, Clovis Ryuichi Nakaie, Maria Verônica Munoz Rojas, Suzana Macedo de Oliveira, Michael Bader, Vânia D'Almeida, Ana Maria Martins, Kely de Picoly Souza, João Bosco Pesquero
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  ABSTRACT: Fabry disease is a multisystem X-linked disorder resulting from α-galactosidase A (α-GalA) gene mutations leading to the accumulation of globotriaosylceramide mainly in endothelium compromising heart, kidney, and brain. In Fabry patients, progressive renal failure is frequently treated with angiotensin I-converting enzyme (ACE) inhibitors. We were interested in the possible interactions between ACE inhibitors therapy and the only causative therapy for Fabry disease, the enzyme replacement therapy (ERT) using recombinant human α-GalA (rhα-GalA). Our results suggest that ACE activity was significantly inhibited in plasma of Fabry patients and the blood pressure level decreased just after ERT (at the end of the rhα-GalA infusion). Interestingly, 2 weeks later, ACE activity was significantly upregulated and the plasma levels of angiotensin II increased in the patients treated with rhα-GalA following the elevations of ACE activity. The same inhibitory effect on ACE activity was also observed in rats after rhα-GalA infusion. Furthermore, ACE activity in CHO cells transfected with the human ACE was inhibited dose and time-dependently by rhα-GalA. In vitro, the incubation of plasma from healthy volunteers with rhα-GalA significantly reduced ACE activity. Finally, rhα-GalA also inhibited ACE activity and released galactose residues from purified rabbit lung ACE dose-dependently. In summary, our results suggest that rhα-GalA interacts with ACE and inhibits its activity, possibly by removing the galactose residues from the enzyme. This modulation might have profound impact on the clinical outcome of Fabry patients treated with rhα-GalA.
  Journal of Molecular Medicine 10/2010; 89(1):65-74. · 4.67 Impact Factor
• Article: Guidelines to diagnosis and monitoring of Fabry disease and review of treatment experiences.

  Ana Maria Martins, Vânia D'Almeida, Sandra Obikawa Kyosen, Edna Tiemi Takata, Alvimar Gonçalves Delgado, Angela Maria Barbosa Ferreira Gonçalves, Caio César Benetti Filho, Dino Martini Filho, Gilson Biagini, Helena Pimentel, [......], Humberto Cenci Guimarães, Jaelson Guilhem Gomes, José Sobral Neto, Luiz Octávio Dias D'Almeida, Luiz Roberto Carvalho, Maria Beatriz Harouche, Maria Cristina Jacometti Maldonado, Osvaldo J M Nascimento, Paulo Sergio dos Santos Montoril, Ricardo Villela Bastos
  The Journal of pediatrics 10/2009; 155(4 Suppl):S19-31. · 4.02 Impact Factor