Altered acetylcholine release in the hippocampus of dystrophin-deficient mice

Parames, S.F.; Coletta-Yudice, E.D.; Nogueira, Felipe Melo; Sousa, M.B. Nering de; Hayashi, Mirian A. F.; Lima‐Landman, Maria Teresa R.; Lapa, Antônio José; Souccar, Caden

doi:10.1016/j.neuroscience.2014.03.050

Cited by 12 publications

(7 citation statements)

References 71 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Activation of nicotinic ACh receptors by ACh (1 mM) or choline (10 mM) enhances the frequency of GABAergic postsynaptic currents in both pyramidal neurons and CA1 interneurons (Alkondon and Albuquerque, 2001). Extended alterations of the cholinergic transmission in mdx hippocampi regarding receptors (Ghedini et al, 2012), ACh release (Parames et al, 2014), and performance in a memory consolidation test (Coccurello et al, 2002) have been described, sustaining the possibility of a presynaptic modulation of GABA release. In addition, some of these structural parameters diverged in 12 months old versus 4 months old mdx mice (Ghedini et al, 2012), thereby suggesting aging-dependent processes.…”

Section: Alterations Due To Lack Of Dystrophinmentioning

confidence: 93%

Acetylcholine, GABA and neuronal networks: A working hypothesis for compensations in the dystrophic brain

Cohen

Quarta

Fulgenzi

et al. 2015

Brain Research Bulletin

View full text Add to dashboard Cite

Section: Alterations Due To Lack Of Dystrophinmentioning

confidence: 93%

Acetylcholine, GABA and neuronal networks: A working hypothesis for compensations in the dystrophic brain

Cohen

Quarta

Fulgenzi

et al. 2015

Brain Research Bulletin

View full text Add to dashboard Cite

“…) and functioning of cholinergic synapses (Parames et al . ). This is reflected in the different cortical phenotypes of the mouse models, with alterations in α‐dystroglycan expression or structure (MORE‐DG, Large myd ) more severe than those seen in the mdx.…”

Section: Age Of Onsetmentioning

confidence: 97%

“…Many studies have shown that the DAPC is involved in cortical development and function [reviewed in (Waite et al 2009)], and this can be seen in these animal models with inactivations in the DAPC as many display central nervous system defects, including the MORE-DG null, Large myd , Large vls , Large enr and the mdx mouse. However, the different components of the DAPC have varying roles in the central nervous system (Waite et al 2009) with a-dystroglycan shown to be important for neuronal migration and dystrophin crucial for postsynaptic clustering of GABAergic receptors (Brunig et al 2002) and functioning of cholinergic synapses (Parames et al 2014). This is reflected in the different cortical phenotypes of the mouse models, with LGMD2D Andersson et al (2012) LGMD2E…”

Section: Age Of Onsetmentioning

confidence: 99%

What do mouse models of muscular dystrophy tell us about the DAPC and its components?

Whitmore

Morgan

2014

Int J Experimental Path

View full text Add to dashboard Cite

SUMMARYThere are over 30 mouse models with mutations or inactivations in the dystrophinassociated protein complex. This complex is thought to play a crucial role in the functioning of muscle, as both a shock absorber and signalling centre, although its role in the pathogenesis of muscular dystrophy is not fully understood. The first mouse model of muscular dystrophy to be identified with a mutation in a component of the dystrophin-associated complex (dystrophin) was the mdx mouse in 1984. Here, we evaluate the key characteristics of the mdx in comparison with other mouse mutants with inactivations in DAPC components, along with key modifiers of the disease phenotype. By discussing the differences between the individual phenotypes, we show that the functioning of the DAPC and consequently its role in the pathogenesis is more complicated than perhaps currently appreciated.

show abstract

“…Nevertheless, most investigators have used mdx mice to dissect the molecular and cellular consequences of dystrophin deficiency in the brain. Collectively, these studies have revealed abnormalities in the hippocampus and in several other regions of the brain ( Ghedini et al, 2012 ; Graciotti et al, 2008 ; Miranda et al, 2011 ; Miranda et al, 2009 ; Parames et al, 2014 ; Vaillend et al, 2004 ; Vaillend et al, 1999 ). So far, only exon skipping has been explored to treat CNS defects.…”

Section: Gene Therapy For Cardiomyopathy and Neuronal Defectsmentioning

confidence: 99%

Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy

McGreevy

Hakim

McIntosh

et al. 2015

Disease Models &Amp; Mechanisms

388

View full text Add to dashboard Cite

Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disorder. It is caused by loss-of-function mutations in the dystrophin gene. Currently, there is no cure. A highly promising therapeutic strategy is to replace or repair the defective dystrophin gene by gene therapy. Numerous animal models of DMD have been developed over the last 30 years, ranging from invertebrate to large mammalian models. mdx mice are the most commonly employed models in DMD research and have been used to lay the groundwork for DMD gene therapy. After ~30 years of development, the field has reached the stage at which the results in mdx mice can be validated and scaled-up in symptomatic large animals. The canine DMD (cDMD) model will be excellent for these studies. In this article, we review the animal models for DMD, the pros and cons of each model system, and the history and progress of preclinical DMD gene therapy research in the animal models. We also discuss the current and emerging challenges in this field and ways to address these challenges using animal models, in particular cDMD dogs.

show abstract

Altered acetylcholine release in the hippocampus of dystrophin-deficient mice

Cited by 12 publications

References 71 publications

Acetylcholine, GABA and neuronal networks: A working hypothesis for compensations in the dystrophic brain

Acetylcholine, GABA and neuronal networks: A working hypothesis for compensations in the dystrophic brain

What do mouse models of muscular dystrophy tell us about the DAPC and its components?

Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy

Contact Info

Product

Resources

About