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Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial

BACKGROUND: Cystic fibrosis (CF) is one of the most common fatal autosomal recessive disorders in the Caucasian population caused by mutations of gene for the cystic fibrosis transmembrane conductance regulator (CFTR). New experimental therapeutic strategies for CF propose a diet supplementation to...

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Autores principales: Scambi, Cinzia, De Franceschi, Lucia, Guarini, Patrizia, Poli, Fabio, Siciliano, Angela, Pattini, Patrizia, Biondani, Andrea, La Verde, Valentina, Bortolami, Oscar, Turrini, Francesco, Carta, Franco, D'Orazio, Ciro, Assael, Baroukh M., Faccini, Giovanni, Bambara, Lisa M.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2652076/
https://www.ncbi.nlm.nih.gov/pubmed/19277125
http://dx.doi.org/10.1371/journal.pone.0004782
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author Scambi, Cinzia
De Franceschi, Lucia
Guarini, Patrizia
Poli, Fabio
Siciliano, Angela
Pattini, Patrizia
Biondani, Andrea
La Verde, Valentina
Bortolami, Oscar
Turrini, Francesco
Carta, Franco
D'Orazio, Ciro
Assael, Baroukh M.
Faccini, Giovanni
Bambara, Lisa M.
author_facet Scambi, Cinzia
De Franceschi, Lucia
Guarini, Patrizia
Poli, Fabio
Siciliano, Angela
Pattini, Patrizia
Biondani, Andrea
La Verde, Valentina
Bortolami, Oscar
Turrini, Francesco
Carta, Franco
D'Orazio, Ciro
Assael, Baroukh M.
Faccini, Giovanni
Bambara, Lisa M.
author_sort Scambi, Cinzia
collection PubMed
description BACKGROUND: Cystic fibrosis (CF) is one of the most common fatal autosomal recessive disorders in the Caucasian population caused by mutations of gene for the cystic fibrosis transmembrane conductance regulator (CFTR). New experimental therapeutic strategies for CF propose a diet supplementation to affect the plasma membrane fluidity and to modulate amplified inflammatory response. The objective of this study was to evaluate the efficacy of 5-methyltetrahydrofolate (5-MTHF) and vitamin B12 supplementation for ameliorating cell plasma membrane features in pediatric patients with cystic fibrosis. METHODOLOGY AND PRINCIPAL FINDINGS: A single arm trial was conducted from April 2004 to March 2006 in an Italian CF care centre. 31 children with CF aged from 3 to 8 years old were enrolled. Exclusion criteria were diabetes, chronic infections of the airways and regular antibiotics intake. Children with CF were supplemented for 24 weeks with 5-methyltetrahydrofolate (5-MTHF, 7.5 mg /day) and vitamin B12 (0.5 mg/day). Red blood cells (RBCs) were used to investigate plasma membrane, since RBCs share lipid, protein composition and organization with other cell types. We evaluated RBCs membrane lipid composition, membrane protein oxidative damage, cation content, cation transport pathways, plasma and RBCs folate levels and plasma homocysteine levels at baseline and after 24 weeks of 5-MTHF and vitamin B12 supplementation. In CF children, 5-MTHF and vitamin B12 supplementation (i) increased plasma and RBC folate levels; (ii) decreased plasma homocysteine levels; (iii) modified RBC membrane phospholipid fatty acid composition; (iv) increased RBC K(+) content; (v) reduced RBC membrane oxidative damage and HSP70 membrane association. CONCLUSION AND SIGNIFICANCE: 5-MTHF and vitamin B12 supplementation might ameliorate RBC membrane features of children with CF. TRIAL REGISTRATION: ClinicalTrials.gov NCT00730509
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spelling pubmed-26520762009-03-11 Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial Scambi, Cinzia De Franceschi, Lucia Guarini, Patrizia Poli, Fabio Siciliano, Angela Pattini, Patrizia Biondani, Andrea La Verde, Valentina Bortolami, Oscar Turrini, Francesco Carta, Franco D'Orazio, Ciro Assael, Baroukh M. Faccini, Giovanni Bambara, Lisa M. PLoS One Research Article BACKGROUND: Cystic fibrosis (CF) is one of the most common fatal autosomal recessive disorders in the Caucasian population caused by mutations of gene for the cystic fibrosis transmembrane conductance regulator (CFTR). New experimental therapeutic strategies for CF propose a diet supplementation to affect the plasma membrane fluidity and to modulate amplified inflammatory response. The objective of this study was to evaluate the efficacy of 5-methyltetrahydrofolate (5-MTHF) and vitamin B12 supplementation for ameliorating cell plasma membrane features in pediatric patients with cystic fibrosis. METHODOLOGY AND PRINCIPAL FINDINGS: A single arm trial was conducted from April 2004 to March 2006 in an Italian CF care centre. 31 children with CF aged from 3 to 8 years old were enrolled. Exclusion criteria were diabetes, chronic infections of the airways and regular antibiotics intake. Children with CF were supplemented for 24 weeks with 5-methyltetrahydrofolate (5-MTHF, 7.5 mg /day) and vitamin B12 (0.5 mg/day). Red blood cells (RBCs) were used to investigate plasma membrane, since RBCs share lipid, protein composition and organization with other cell types. We evaluated RBCs membrane lipid composition, membrane protein oxidative damage, cation content, cation transport pathways, plasma and RBCs folate levels and plasma homocysteine levels at baseline and after 24 weeks of 5-MTHF and vitamin B12 supplementation. In CF children, 5-MTHF and vitamin B12 supplementation (i) increased plasma and RBC folate levels; (ii) decreased plasma homocysteine levels; (iii) modified RBC membrane phospholipid fatty acid composition; (iv) increased RBC K(+) content; (v) reduced RBC membrane oxidative damage and HSP70 membrane association. CONCLUSION AND SIGNIFICANCE: 5-MTHF and vitamin B12 supplementation might ameliorate RBC membrane features of children with CF. TRIAL REGISTRATION: ClinicalTrials.gov NCT00730509 Public Library of Science 2009-03-11 /pmc/articles/PMC2652076/ /pubmed/19277125 http://dx.doi.org/10.1371/journal.pone.0004782 Text en Scambi et al. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
Scambi, Cinzia
De Franceschi, Lucia
Guarini, Patrizia
Poli, Fabio
Siciliano, Angela
Pattini, Patrizia
Biondani, Andrea
La Verde, Valentina
Bortolami, Oscar
Turrini, Francesco
Carta, Franco
D'Orazio, Ciro
Assael, Baroukh M.
Faccini, Giovanni
Bambara, Lisa M.
Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial
title Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial
title_full Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial
title_fullStr Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial
title_full_unstemmed Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial
title_short Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial
title_sort preliminary evidence for cell membrane amelioration in children with cystic fibrosis by 5-mthf and vitamin b12 supplementation: a single arm trial
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2652076/
https://www.ncbi.nlm.nih.gov/pubmed/19277125
http://dx.doi.org/10.1371/journal.pone.0004782
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