RETRACTED ARTICLE: Neurotrophic effects of Cerebrolysin in the Mecp2308/Y transgenic model of Rett syndrome
- 4 July 2008
- journal article
- retracted article
- Published by Springer Nature in Acta Neuropathologica
- Vol. 116 (4) , 425-437
Abstract
Rett syndrome is a childhood neurodevelopmental disorder caused by mutations in the gene encoding for methyl-CpG-binding protein (MeCP2). Neuropathological studies in patients with Rett syndrome and in MeCP2 mutant models have shown reduced dendritic arborization and abnormal neuronal packing. We have previously shown that Cerebrolysin (CBL), a neurotrophic peptide mixture, ameliorates the synaptic and dendritic pathology in models of aging and neurodegeneration. This study aimed to determine whether CBL was capable of reducing behavioral and neuronal alterations in Mecp2308/Y mutant mice. Two sets of experiments were performed, the first with 4-month-old male Mecp2308/Y mutant mice treated with CBL or vehicle for 3 months (Group A) and the second with 1-month-old mice treated for 6 months (Group B). Behavioral analysis showed improved motor performance with CBL in Group A and a trend toward improvement in Group B. Consistent with behavioral findings, neuropathological analysis of the basal ganglia showed amelioration of dendritic simplification in CBL-treated Mecp2308/Y mutant mice. CBL treatment also ameliorated dendritic pathology and neuronal loss in the hippocampus and neocortex in Mecp2308/Y mutant mice. In conclusion, this study demonstrates that CBL promotes recovery of dendritic and neuronal damage and behavioral improvements in young adult Mecp2308/Y mutant mice and suggests that CBL may have neurotrophic effects in this model. These findings support the possibility that CBL may have beneficial effects in the management of Rett syndrome.Keywords
This publication has 67 references indexed in Scilit:
- Escalating dose-multiple binge methamphetamine exposure results in degeneration of the neocortex and limbic system in the ratExperimental Neurology, 2007
- Meta-analysis: the efficacy of nootropic agent Cerebrolysin in the treatment of Alzheimer’s diseaseJournal of Neural Transmission, 2007
- Cerebrolysin decreases amyloid‐β production by regulating amyloid protein precursor maturation in a transgenic model of Alzheimer's diseaseJournal of Neuroscience Research, 2006
- Abnormalities of social interactions and home-cage behavior in a mouse model of Rett syndromeHuman Molecular Genetics, 2004
- Rett Syndrome - an updateJournal of Neural Transmission, 2003
- Rescue of rat spinal motoneurons from avulsion-induced cell death by intrathecal administration of IGF-I and CerebrolysinAnnals of Anatomy - Anatomischer Anzeiger, 2003
- Effects of Cerebrolysin on the outgrowth and protection of processes of cultured brain neuronsJournal of Neural Transmission, 2001
- Antiapoptotic effects of the peptidergic drug Cerebrolysin on primary cultures of embryonic chick cortical neuronsJournal of Neural Transmission, 2001
- Purification, sequence, and cellular localization of a novel chromosomal protein that binds to Methylated DNACell, 1992
- Dendrites, dementia and the down syndromeBrain and Development, 1989