Regular ArticleDystrophin Stabilizes α3- But Not α7-Containing Nicotinic Acetylcholine Receptor Subtypes at the Postsynaptic Apparatus in the Mouse Superior Cervical Ganglion
References (56)
- et al.
Antibodies against neuronal nicotinic receptor subtypes are present in patients with neurological disorders
J. Neuroimmunol.
(2000) - et al.
Duchenne muscular dystrophy: Deficiency of dystrophin at the muscle cell surface
Cell
(1988) - et al.
Neuronal nicotinic receptors, important new players in brain function
Eur. J. Pharmacol.
(2000) - et al.
Dystrophin and its isoforms in a sympathetic ganglion of normal and dystrophic mdx mice: Immunolocalization by electron microscopy and biochemical characterization
Neuroscience
(1997) - et al.
Anti-neuronal nicotinic receptor antibodies in MG patients with thymoma
J. Neuroimmunol.
(2001) - et al.
Dystroglycan: An extracellular matrix receptor linked to the cytoskeleton
Curr. Opin. Cell Biol.
(1996) - et al.
Dystrophin: The protein product of the Duchenne muscular dystrophy locus
Cell
(1987) - et al.
Heteromultimerization and NMDA receptor-clustering activity of chapsin-110, a member of the PSD-95 family of proteins
Neuron
(1996) - et al.
The ultrastructural distribution of putative nicotinic receptors on cultured neurons from the rat superior cervical ganglion
Neuroscience
(1988) - et al.
LIGAND: a versatile computerized approach for characterization of ligand-binding systems
Anal. Biochem.
(1980)
Dystroglycan distribution in adult mouse brain: a light and electron microscopy study
Neuroscience
The structural and functional diversity of dystrophin
Nature Genet.
Ultrastructural localization of the α4-subunit of the neuronal acetylcholine nicotinic receptor in the rat substantia nigra
J. Neurosci.
Chick optic lobe contains a developmentally regulated α2α5β2 nicotinic receptor subtype
Mol. Pharmacol.
Homer: A protein that selectively binds metabotropic glutamate receptors
Nature
Host cell-specific folding and assembly of the neuronal nicotinic acetylcholine receptor α7 subunit
J. Neurochem.
Nex mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin
Nature Genet.
Two distinct classes of functional α7-containing nicotinic receptor on rat superior cervical neurons
J. Physiol.
Antagonism of nicotinic receptors of rat chromaffin cells by N,N,N-trimethyl-1-(4-trans-stilbenoxy)-2-propylammonium iodide: A patch clamp and ligand binding study
Br. J. Pharmacol.
GRIP: A synaptic PDZ domain-containing protein that interacts vith AMPA receptors
Nature
Neuronal α-Bungarotoxin receptors are α7 subunit homomers
J. Neurosci.
Duchenne Muscular Dystrophy
Postsynaptic clustering of major GABAA receptor subtypes requires the gamma-2 subunit and gephyrin
Nature Neurosci.
Alternative splicing of human dystrophin mRNA generates isoforms at the carboxy terminus
Nature
Dual requirement for gephyrin in glycine receptor clustering and molybdoenzyme activity
Science
Comparative pharmacology of epibatidine: A potent agonist for neuronal nicotinic acetylcholine receptors
Mol. Pharmacol.
Specific expression of G-dystrophin (Dp71) in the brain
NeuroReport
Cited by (22)
Synaptic alterations as a neurodevelopmental trait of Duchenne muscular dystrophy
2022, Neurobiology of DiseaseCitation Excerpt :Differently from muscular degeneration, behavioral and neurological complications associated to DMD are not progressive, indicating that the underlying neuroanatomical, molecular, and functional alterations arise at fetal stages and at birth, thus strictly related to neurodevelopment. The lack of Dp427 alone affects development (Sbriccoli et al., 1995; Carretta et al., 2001; De Stefano et al., 2005; Licursi et al., 2012; Lombardi et al., 2017; Persiconi et al., 2020), connectivity (Vaillend et al., 1999a, 1999b; Del Signore et al., 2002; Anderson et al., 2003; Vaillend and Billard, 2002; Vaillend et al., 2004; Briatore et al., 2020;), and physiology (i.e. cognition, behavior, stress response) (Mehler et al., 1992; Mehler and Kessler, 1998; Vaillend et al., 1999a, 1999b; Anderson et al., 2002; Coccurello et al., 2002; Vaillend et al., 2002; Vaillend et al., 2004; Di Angelantonio et al., 2011; Fragapane et al., 2020; Comim et al., 2019; Caudal et al., 2020) of selected autonomic and central neurons. Particularly affected are neural circuits involved in the processing of fear response, as lack of full-length dystrophin in mdx mice significantly enhances fearfulness associated to mild stress and induces delayed fear learning and memory (Goyenvalle et al., 2015; Razzoli et al., 2020; Sekiguchi et al., 2009; Vaillend and Chaussenot, 2017).
Quantitative changes of nicotinic receptors in the hippocampus of dystrophin-deficient mice
2012, Brain ResearchCitation Excerpt :The lack of dystrophin and DGC disrupts the cell membrane structure, resulting in a reduction in the number and size of GABAAR clusters in the hippocampus and cerebellum, where the protein is colocalized with these receptors (Graciotti et al., 2008; Knuesel et al., 1999). Decrease in the α3β2/β4 subtypes of nAChR in the superior cervical ganglion (Del Signore et al., 2002; Di Angelantonio et al., 2011), fragmentation of the neuromuscular junction, and upregulation of the embryonic-type nAChRs have also been reported in dystrophin-deficient mice (Ghedini et al., 2008; Grady et al., 2000; Huh and Fuhrer, 2002). Thus, it seems conceivable that the structural disorganization of the cell membrane, associated with a lack of dystrophin, might also be involved in the altered number of [3H]-cytisine and [125I]-αBGT binding sites observed in the hippocampus of mdx mice.
Lack of dystrophin functionally affects α3β2/β4-nicotinic acethylcholine receptors in sympathetic neurons of dystrophic mdx mice
2011, Neurobiology of DiseaseCitation Excerpt :We previously demonstrated that neurons of the mouse and rat sympathetic superior cervical ganglion (SCG) express nAChRs containing the α3 subunit, associated with β2 and/or β4 (α3β2/β4-nAChRs), and nAChRs containing the α7 subunit (α7-nAChR) (Del Signore et al., 2002, 2004). Moreover, combining pharmacological and immunohistochemical studies, we demonstrated that only α3β2/β4-nAChRs are stabilized by dystrophin (Del Signore et al., 2002), which in wild-type mice is localized at the post-synaptic apparatus of several intra-ganglionic synapses (De Stefano et al., 1997; Zaccaria et al., 1998). In fact, with respect to the wild-type, in SCG neurons of mdx mice, lack of dystrophin causes a decrease in α3β2/β4-nAChRs, but not in α7-nAChRs, leaving unaffected the ligand-binding properties of both receptors (Del Signore et al., 2002).
Diversity of vertebrate nicotinic acetylcholine receptors
2009, NeuropharmacologyComponents of the NGF signaling complex are altered in mdx mouse superior cervical ganglion and its target organs
2008, Neurobiology of DiseaseDystrophin and utrophin isoforms are expressed in glia, but not neurons, of the avian parasympathetic ciliary ganglion
2008, Brain ResearchCitation Excerpt :Dystrophin colocalizes with selective GABAA receptor subunits at a subset of inhibitory GABAergic synapses in the CNS (Knuesel et al., 1999, 2001; Fritschy et al., 2003). Dystrophin also localizes to a subset of α3-nAChR-rich postsynaptic sites in sympathetic superior cervical ganglion (SCG) neurons (De Stefano et al., 1997; Zaccaria et al., 2000; Del Signore et al., 2002). Although dystrophin is not required for cluster formation, there are decreases in the number of GABAA receptor and α3-containing nAChR clusters and impaired inhibitory synaptic transmission in mdx and dystrobrevin mutant mice (Levi et al., 2002; Knuesel et al., 1999; Zaccaria et al., 2000; Del Signore et al., 2002; Anderson et al., 2003; Grady et al., 2006; Kueh et al., 2008).
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Both authors contributed equally to this work.