RT Journal Article
T1 Melatonin Treatment Reduces Oxidative Damage and Normalizes Plasma Pro-Inflammatory Cytokines in Patients Suffering from Charcot-Marie-Tooth Neuropathy: A Pilot Study in Three Children
A1 Chahbouni, Mariam
A1 del Senor Lopez, Maria
A1 Molina-Carballo, Antonio
A1 de Haro, Tomas
A1 Munoz-Hoyos, Antonio
A1 Fernandez-Ortiz, Marisol
A1 Guerra-Librero, Ana
A1 Acuna-Castroviejo, Dario
K1 antioxidant
K1 cytokines
K1 inflammation
K1 melatonin therapy
K1 oxidative stress
K1 axonal neuropathy
K1 Glutathione s-transferases
K1 Lipid-peroxidation
K1 Skeletal-muscle
K1 Mitochondrial dysfunction
K1 Cmt disease
K1 Stress
K1 Antioxidant
K1 Expression
K1 Molecule
K1 Polyneuropathy
AB Charcot-Marie-Tooth neuropathy (CMT) is a motor and sensory neuropathy comprising a heterogeneous group of inherited diseases. The CMT1A phenotype is predominant in the 70% of CMT patients, with nerve conduction velocity reduction and hypertrophic demyelination. These patients have elevated oxidative stress and chronic inflammation. Currently, there is no effective cure for CMT; herein, we investigated whether melatonin treatment may reduce the inflammatory and oxidative damage in CMT1A patients. Three patients, aged 8-10 years, were treated with melatonin (60 mg at 21:00 h plus 10 mg at 09:00 h), and plasma levels of lipid peroxidation (LPO), nitrites (NOx), IL-1 beta, IL-2, IL-6, TNF-alpha, INF-gamma, oxidized to reduced glutathione (GSSG/GSH) ratio, and the activities of superoxide dismutase (SOD), glutathione-S transferase (GST), glutathione peroxidase (GPx), and reductase (GRd), were determined in erythrocytes at 3 and 6 months of treatment. Healthy age- and sex-matched subjects were used as controls. The results showed increased activities of SOD, GST, GPx, and GRd in CMT1A patients, which were reduced at 3 and 6 months of treatment. The GSSG/GSH ratio significantly increased in the patients, returning to control values after melatonin treatment. The inflammatory process was confirmed by the elevation of all proinflammatory cytokines measured, which were also normalized by melatonin. LPO and NOx, which also were elevated in the patients, were normalized by melatonin. The results document beneficial effects of the use of melatonin in CMT1A patients to reduce the hyperoxidative and inflammatory condition, which may correlate with a reduction of the degenerative process.
PB Mdpi
SN 1420-3049
YR 2017
FD 2017-10-01
LK http://hdl.handle.net/10668/19306
UL http://hdl.handle.net/10668/19306
LA en
DS RISalud
RD Apr 7, 2025