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Andrea Nistri - One of the best experts on this subject based on the ideXlab platform.
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inefficient constitutive inhibition of p2x3 receptors by brain natriuretic peptide system contributes to sensitization of trigeminal sensory neurons in a Genetic mouse model of familial hemiplegic migraine
Molecular Pain, 2016Co-Authors: Anna Marchenkova, Sandra Vilotti, Niels R Ntamati, Arn M J M Van Den Maagdenberg, Andrea NistriAbstract:BackgroundOn trigeminal ganglion neurons, pain-sensing P2X3 receptors are constitutively inhibited by brain natriuretic peptide via its natriuretic peptide receptor-A. This inhibition is associated with increased P2X3 serine phosphorylation and receptor redistribution to non-lipid raft membrane compartments. The natriuretic peptide receptor-A antagonist anantin reverses these effects. We studied whether P2X3 inhibition is dysfunctional in a Genetic familial hemiplegic migraine type-1 model produced by introduction of the human pathogenic R192Q missense mutation into the mouse CACNA1A Gene (Knock-In phenotype). This model faithfully replicates several properties of familial hemiplegic migraine type-1, with gain-of-function of CaV2.1 Ca2+ channels, raised levels of the algogenic peptide calcitonin Gene-related peptide, and enhanced activity of P2X3 receptors in trigeminal ganglia.ResultsIn Knock-In neurons, anantin did not affect P2X3 receptor activity, membrane distribution, or serine phosphorylation level...
Anna Marchenkova - One of the best experts on this subject based on the ideXlab platform.
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inefficient constitutive inhibition of p2x3 receptors by brain natriuretic peptide system contributes to sensitization of trigeminal sensory neurons in a Genetic mouse model of familial hemiplegic migraine
Molecular Pain, 2016Co-Authors: Anna Marchenkova, Sandra Vilotti, Niels R Ntamati, Arn M J M Van Den Maagdenberg, Andrea NistriAbstract:BackgroundOn trigeminal ganglion neurons, pain-sensing P2X3 receptors are constitutively inhibited by brain natriuretic peptide via its natriuretic peptide receptor-A. This inhibition is associated with increased P2X3 serine phosphorylation and receptor redistribution to non-lipid raft membrane compartments. The natriuretic peptide receptor-A antagonist anantin reverses these effects. We studied whether P2X3 inhibition is dysfunctional in a Genetic familial hemiplegic migraine type-1 model produced by introduction of the human pathogenic R192Q missense mutation into the mouse CACNA1A Gene (Knock-In phenotype). This model faithfully replicates several properties of familial hemiplegic migraine type-1, with gain-of-function of CaV2.1 Ca2+ channels, raised levels of the algogenic peptide calcitonin Gene-related peptide, and enhanced activity of P2X3 receptors in trigeminal ganglia.ResultsIn Knock-In neurons, anantin did not affect P2X3 receptor activity, membrane distribution, or serine phosphorylation level...
John H Weiss - One of the best experts on this subject based on the ideXlab platform.
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slow development of als like spinal cord pathology in mutant valosin containing protein Gene knock in mice
Cell Death and Disease, 2012Co-Authors: Hong Z Yin, Angele Nalbandian, Katrina J Llewellyn, Tahseen Mozaffar, Virginia Kimonis, C I Hsu, John H WeissAbstract:Pathological features of amyotrophic lateral sclerosis (ALS) include, in addition to selective motor neuron (MN) deGeneration, the occurrence of protein aggregates, mitochondrial dysfunction and astrogliosis. SOD1 mutations cause rare familial forms of ALS and have provided the most widely studied animal models. Relatively recent studies implicating another protein, TDP-43, in familial and sporadic forms of ALS have led to the development of new animal models. More recently, mutations in the valosin-containing protein (VCP) Gene linked to the human Genetic disease, Inclusion Body Myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD), were found also to be associated with ALS in some patients. A heterozygous Knock-In VCP mouse model of IBMPFD (VCP(R155H/+)) exhibited muscle, bone and brain pathology characteristic of the human disease. We have undertaken studies of spinal cord pathology in VCP(R155H/+) mice and find age-dependent deGeneration of ventral horn MNs, TDP-43-positive cytosolic inclusions, mitochondrial aggregation and progressive astrogliosis. Aged animals (~24-27 months) show electromyography evidence of denervation consistent with the observed MN loss. Although these animals do not develop rapidly progressive fatal ALS-like disease during their lifespans, they recapitulate key pathological features of both human disease and other animal models of ALS, and may provide a valuable new model for studying events preceding onset of catastrophic disease.
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slow development of als like spinal cord pathology in mutant valosin containing protein Gene knock in mice
Cell Death and Disease, 2012Co-Authors: Angele Nalbandian, S Li, Katrina J Llewellyn, Tahseen Mozaffar, Virginia Kimonis, John H WeissAbstract:Pathological features of amyotrophic lateral sclerosis (ALS) include, in addition to selective motor neuron (MN) deGeneration, the occurrence of protein aggregates, mitochondrial dysfunction and astrogliosis. SOD1 mutations cause rare familial forms of ALS and have provided the most widely studied animal models. Relatively recent studies implicating another protein, TDP-43, in familial and sporadic forms of ALS have led to the development of new animal models. More recently, mutations in the valosincontaining protein (VCP) Gene linked to the human Genetic disease, Inclusion Body Myopathy associated with Paget’s disease of bone and frontotemporal dementia (IBMPFD), were found also to be associated with ALS in some patients. A heterozygous Knock-In VCP mouse model of IBMPFD (VCP R155H/ þ ) exhibited muscle, bone and brain pathology characteristic of the human disease. We have undertaken studies of spinal cord pathology in VCP R155H/ þ mice and find age-dependent deGeneration of ventral horn MNs, TDP-43-positive cytosolic inclusions, mitochondrial aggregation and progressive astrogliosis. Aged animals (B24–27 months) show electromyography evidence of denervation consistent with the observed MN loss. Although these animals do not develop rapidly progressive fatal ALS-like disease during their lifespans, they recapitulate key pathological features of both human disease and other animal models of ALS, and may provide a valuable new model for studying events preceding onset of catastrophic disease. Cell Death and Disease (2012) 3, e374; doi:10.1038/cddis.2012.115; published online 16 August 2012 Subject Category: Neuroscience Amyotrophic lateral sclerosis (ALS) is characterized by a number of pathological hallmarks, including selective motor neuron (MN) loss, intracellular inclusions, astrogliosis, and mitochondrial abnormalities. 1 Whereas most cases are sporadic, B10% are familial and some of those are caused by mutations in the SOD1 Gene. Although transgenic mutant SOD1 animals, which provide the most widely studied models of the disease, display many of these features, 1,2 therapeutic efficacy in these animals has been poorly predictive of human efficacy, and it is not clear the degree to which studies in these models overexpressing a Gene implicated in a small subset of human cases will prove applicable to human disease. An important breakthrough came with the relatively recent findings implicating roles of an unrelated protein, TDP-43 in familial as well as sporadic ALS. 3,4 TDP-43 containing inclusions are found in both forms of the disease, and mutations in TDP-43 are associated with some familial forms of ALS. Interestingly, TDP-43 pathology is also associated with forms of frontotemporal dementia (FTD), suggesting some mechanistic overlap between these neurodeGenerative diseases. 3‐6 Elucidation of the link between TDP-43 and ALS has led to the recent development of some new animal models. 7 Although the mechanism of injury is still unclear, 8 TDP-43 is normally a nuclear RNA/DNA-binding protein involved in multiple aspects of RNA metabolism, and, in disease, often appears in cytosolic inclusions. Suggesting a broad relevance of consequent mechanisms, a related RNA/DNA-binding protein, fused in sarcoma (FUS), is also associated with forms of FTD and ALS. 6 Mutations in an unrelated Gene, valosin-containing protein (VCP), were previously found to be associated with a Genetic disease, Inclusion Body Myopathy associated with Paget’s disease of bone and frontotemporal dementia (IBMPFD). 9
Sandra Vilotti - One of the best experts on this subject based on the ideXlab platform.
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inefficient constitutive inhibition of p2x3 receptors by brain natriuretic peptide system contributes to sensitization of trigeminal sensory neurons in a Genetic mouse model of familial hemiplegic migraine
Molecular Pain, 2016Co-Authors: Anna Marchenkova, Sandra Vilotti, Niels R Ntamati, Arn M J M Van Den Maagdenberg, Andrea NistriAbstract:BackgroundOn trigeminal ganglion neurons, pain-sensing P2X3 receptors are constitutively inhibited by brain natriuretic peptide via its natriuretic peptide receptor-A. This inhibition is associated with increased P2X3 serine phosphorylation and receptor redistribution to non-lipid raft membrane compartments. The natriuretic peptide receptor-A antagonist anantin reverses these effects. We studied whether P2X3 inhibition is dysfunctional in a Genetic familial hemiplegic migraine type-1 model produced by introduction of the human pathogenic R192Q missense mutation into the mouse CACNA1A Gene (Knock-In phenotype). This model faithfully replicates several properties of familial hemiplegic migraine type-1, with gain-of-function of CaV2.1 Ca2+ channels, raised levels of the algogenic peptide calcitonin Gene-related peptide, and enhanced activity of P2X3 receptors in trigeminal ganglia.ResultsIn Knock-In neurons, anantin did not affect P2X3 receptor activity, membrane distribution, or serine phosphorylation level...
Niels R Ntamati - One of the best experts on this subject based on the ideXlab platform.
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inefficient constitutive inhibition of p2x3 receptors by brain natriuretic peptide system contributes to sensitization of trigeminal sensory neurons in a Genetic mouse model of familial hemiplegic migraine
Molecular Pain, 2016Co-Authors: Anna Marchenkova, Sandra Vilotti, Niels R Ntamati, Arn M J M Van Den Maagdenberg, Andrea NistriAbstract:BackgroundOn trigeminal ganglion neurons, pain-sensing P2X3 receptors are constitutively inhibited by brain natriuretic peptide via its natriuretic peptide receptor-A. This inhibition is associated with increased P2X3 serine phosphorylation and receptor redistribution to non-lipid raft membrane compartments. The natriuretic peptide receptor-A antagonist anantin reverses these effects. We studied whether P2X3 inhibition is dysfunctional in a Genetic familial hemiplegic migraine type-1 model produced by introduction of the human pathogenic R192Q missense mutation into the mouse CACNA1A Gene (Knock-In phenotype). This model faithfully replicates several properties of familial hemiplegic migraine type-1, with gain-of-function of CaV2.1 Ca2+ channels, raised levels of the algogenic peptide calcitonin Gene-related peptide, and enhanced activity of P2X3 receptors in trigeminal ganglia.ResultsIn Knock-In neurons, anantin did not affect P2X3 receptor activity, membrane distribution, or serine phosphorylation level...