2021
DOI: 10.3390/ijms22136739
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Treatment with the Bacterial Toxin CNF1 Selectively Rescues Cognitive and Brain Mitochondrial Deficits in a Female Mouse Model of Rett Syndrome Carrying a MeCP2-Null Mutation

Abstract: Rett syndrome (RTT) is a rare neurological disorder caused by mutations in the X-linked MECP2 gene and a major cause of intellectual disability in females. No cure exists for RTT. We previously reported that the behavioural phenotype and brain mitochondria dysfunction are widely rescued by a single intracerebroventricular injection of the bacterial toxin CNF1 in a RTT mouse model carrying a truncating mutation of the MeCP2 gene (MeCP2-308 mice). Given the heterogeneity of MECP2 mutations in RTT patients, we te… Show more

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Cited by 7 publications
(8 citation statements)
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“…Through these effects, CNF1 modulates actin cytoskeleton dynamics and enhances synaptic transmission and plasticity (Amir et al, 1999;Diana et al, 2007). Mecp2 KO and HET mice treated with CNF1 show a significant improvement in cognition and motor function (308/Y and Bird line) (De Filippis et al, 2012;Urbinati et al, 2021). CNF1 treatment also prevents an enhancement of GluN2B-tyrosine phosphorylation and facilitates LTP induction in hippocampal CA1 of Mecp2 HET mice.…”
Section: Mitochondria Targeting Drugsmentioning
confidence: 99%
“…Through these effects, CNF1 modulates actin cytoskeleton dynamics and enhances synaptic transmission and plasticity (Amir et al, 1999;Diana et al, 2007). Mecp2 KO and HET mice treated with CNF1 show a significant improvement in cognition and motor function (308/Y and Bird line) (De Filippis et al, 2012;Urbinati et al, 2021). CNF1 treatment also prevents an enhancement of GluN2B-tyrosine phosphorylation and facilitates LTP induction in hippocampal CA1 of Mecp2 HET mice.…”
Section: Mitochondria Targeting Drugsmentioning
confidence: 99%
“…It is interesting to note that the modulation of Rho GTPases by CNF1 has been described to rescue mitochondrial dysfunctions in Mecp2‐308 mice and null mice 12,25 . Various lines of evidence point to mitochondria dysfunction as a possible contributing factor in RTT pathogenesis.…”
Section: Discussionmentioning
confidence: 99%
“…Several metabolic components contribute to the phenotypical manifestations of RTT, 16 , 35 thus making difficult the analysis of RTT development and progression. Rho GTPases modulation by the bacterial product CNF1 has been reported to ameliorate the cognitive deficits and to rescue the mitochondrial defects in in vivo models of RTT, 12 , 18 , 25 thus suggesting CNF1 as a promising therapeutic drug. However, the molecular and cellular mechanisms which underlie the beneficial effects observed in the RTT models after exposure to CNF1 were still unclear.…”
Section: Discussionmentioning
confidence: 99%
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