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Serum of Mothers Having Autistic Children Induces Cerebellar Purkinje cell Alterations in Experimental Model: A Possible Cause of Autism / Silva G. KOURTIAN in Autism - Open Access, 5-2 ([01/03/2015])
[article]
Titre : Serum of Mothers Having Autistic Children Induces Cerebellar Purkinje cell Alterations in Experimental Model: A Possible Cause of Autism Type de document : Texte imprimé et/ou numérique Auteurs : Silva G. KOURTIAN, Auteur ; Nabila E. ABDELMEGUID, Auteur Article en page(s) : 7 p. Langues : Anglais (eng) Mots-clés : Autism Purkinje neurons Plasma cells Rattus Norvegicus Index. décimale : PER Périodiques Résumé : Autism is a brain developmental disorder characterized by impaired social interaction. Our aim is to assess the morphology of plasma cells of mothers having autistic children and to evaluate the effect of administration of serum obtained from mothers having autistic children on the cerebellar Purkinje neurons. Two groups were used: control group, where pregnant rats remained intact while others were injected intraperitoneally with serum of mothers with no history of autism. In the experimental group pregnant rats were intraperitoneally injected serum of autistic children's mothers. The Transmission Electron Microscope showed altered plasma cells of autistic children's mothers, where the heterochromatin material had inversed distribution in all nuclei examined in addition to other alterations. Also, modified Purkinje neurons were obtained in neonates of experimental group showing degeneration and atrophied dendrites. Recommended examination of peripheral blood is essential for early intervention. En ligne : https://dx.doi.org/10.4172/2165-7890.1000142 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=409
in Autism - Open Access > 5-2 [01/03/2015] . - 7 p.[article] Serum of Mothers Having Autistic Children Induces Cerebellar Purkinje cell Alterations in Experimental Model: A Possible Cause of Autism [Texte imprimé et/ou numérique] / Silva G. KOURTIAN, Auteur ; Nabila E. ABDELMEGUID, Auteur . - 7 p.
Langues : Anglais (eng)
in Autism - Open Access > 5-2 [01/03/2015] . - 7 p.
Mots-clés : Autism Purkinje neurons Plasma cells Rattus Norvegicus Index. décimale : PER Périodiques Résumé : Autism is a brain developmental disorder characterized by impaired social interaction. Our aim is to assess the morphology of plasma cells of mothers having autistic children and to evaluate the effect of administration of serum obtained from mothers having autistic children on the cerebellar Purkinje neurons. Two groups were used: control group, where pregnant rats remained intact while others were injected intraperitoneally with serum of mothers with no history of autism. In the experimental group pregnant rats were intraperitoneally injected serum of autistic children's mothers. The Transmission Electron Microscope showed altered plasma cells of autistic children's mothers, where the heterochromatin material had inversed distribution in all nuclei examined in addition to other alterations. Also, modified Purkinje neurons were obtained in neonates of experimental group showing degeneration and atrophied dendrites. Recommended examination of peripheral blood is essential for early intervention. En ligne : https://dx.doi.org/10.4172/2165-7890.1000142 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=409 Cerebellar demyelination and neurodegeneration associated with mTORC1 hyperactivity may contribute to the developmental onset of autism-like neurobehavioral phenotype in a rat model / Viera KUTNA in Autism Research, 15-5 (May 2022)
[article]
Titre : Cerebellar demyelination and neurodegeneration associated with mTORC1 hyperactivity may contribute to the developmental onset of autism-like neurobehavioral phenotype in a rat model Type de document : Texte imprimé et/ou numérique Auteurs : Viera KUTNA, Auteur ; Valerie BRID O'LEARY, Auteur ; Cyril HOSCHL, Auteur ; Saak V. OVSEPIAN, Auteur Article en page(s) : p.791-805 Langues : Anglais (eng) Mots-clés : Animals Autism Spectrum Disorder Autistic Disorder Cerebellum/metabolism Demyelinating Diseases/complications/metabolism Epilepsy/complications Humans Mechanistic Target of Rapamycin Complex 1/genetics/metabolism Phenotype Rats Tuberous Sclerosis Purkinje neurons cerebellum demyelination mTORC1 signaling microglia activation synaptophysin Index. décimale : PER Périodiques Résumé : The cerebellum hosts more than half of all neurons of the human brain, with their organized activity playing a key role in coordinating motor functions. Cerebellar activity has also been implicated in the control of speech, communication, and social behavior, which are compromised in autism spectrum disorders (ASD). Despite major research advances, there is a shortage of mechanistic data relating cellular and molecular changes in the cerebellum to autistic behavior. We studied the impact of tuberous sclerosis complex 2 haploinsufficiency (Tsc2+/-) with downstream mTORC1 hyperactivity on cerebellar morphology and cellular organization in 1, 9, and 18?m.o. Eker rats, to determine possible structural correlates of an autism-like behavioural phenotype in this model. We report a greater developmental expansion of the cerebellar vermis, owing to enlarged white matter and thickened molecular layer. Histochemical and immunofluorescence data suggest age-related demyelination of central tract of the vermis, as evident from reduced level of myelin-basic protein in the arbora vitae. We also observed a higher number of astrocytes in Tsc2+/- rats of older age while the number of Purkinje cells (PCs) in these animals was lower than in wild-type controls. Unlike astrocytes and PCs, Bergmann glia remained unaltered at all ages in both genotypes, while the number of microglia was higher in Tsc2+/- rats of older age. The convergent evidence for a variety of age-dependent cellular changes in the cerebellum of rats associated with mTORC1 hyperactivity, thus, predicts an array of functional impairments, which may contribute to the developmental onset of an autism-like behavioral phenotype in this model. LAY SUMMARY: This study elucidates the impact of constitutive mTORC1 hyperactivity on cerebellar morphology and cellular organization in a rat model of autism and epilepsy. It describes age-dependent degeneration of Purkinje neurons, with demyelination of central tract as well as activation of microglia, and discusses the implications of these changes for neuro-behavioral phenotypes. The described changes provide new indications for the putative mechanisms underlying cerebellar impairments with their age-related onset, which may contribute to the pathobiology of autism, epilepsy, and related disorders. En ligne : http://dx.doi.org/10.1002/aur.2688 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=473
in Autism Research > 15-5 (May 2022) . - p.791-805[article] Cerebellar demyelination and neurodegeneration associated with mTORC1 hyperactivity may contribute to the developmental onset of autism-like neurobehavioral phenotype in a rat model [Texte imprimé et/ou numérique] / Viera KUTNA, Auteur ; Valerie BRID O'LEARY, Auteur ; Cyril HOSCHL, Auteur ; Saak V. OVSEPIAN, Auteur . - p.791-805.
Langues : Anglais (eng)
in Autism Research > 15-5 (May 2022) . - p.791-805
Mots-clés : Animals Autism Spectrum Disorder Autistic Disorder Cerebellum/metabolism Demyelinating Diseases/complications/metabolism Epilepsy/complications Humans Mechanistic Target of Rapamycin Complex 1/genetics/metabolism Phenotype Rats Tuberous Sclerosis Purkinje neurons cerebellum demyelination mTORC1 signaling microglia activation synaptophysin Index. décimale : PER Périodiques Résumé : The cerebellum hosts more than half of all neurons of the human brain, with their organized activity playing a key role in coordinating motor functions. Cerebellar activity has also been implicated in the control of speech, communication, and social behavior, which are compromised in autism spectrum disorders (ASD). Despite major research advances, there is a shortage of mechanistic data relating cellular and molecular changes in the cerebellum to autistic behavior. We studied the impact of tuberous sclerosis complex 2 haploinsufficiency (Tsc2+/-) with downstream mTORC1 hyperactivity on cerebellar morphology and cellular organization in 1, 9, and 18?m.o. Eker rats, to determine possible structural correlates of an autism-like behavioural phenotype in this model. We report a greater developmental expansion of the cerebellar vermis, owing to enlarged white matter and thickened molecular layer. Histochemical and immunofluorescence data suggest age-related demyelination of central tract of the vermis, as evident from reduced level of myelin-basic protein in the arbora vitae. We also observed a higher number of astrocytes in Tsc2+/- rats of older age while the number of Purkinje cells (PCs) in these animals was lower than in wild-type controls. Unlike astrocytes and PCs, Bergmann glia remained unaltered at all ages in both genotypes, while the number of microglia was higher in Tsc2+/- rats of older age. The convergent evidence for a variety of age-dependent cellular changes in the cerebellum of rats associated with mTORC1 hyperactivity, thus, predicts an array of functional impairments, which may contribute to the developmental onset of an autism-like behavioral phenotype in this model. LAY SUMMARY: This study elucidates the impact of constitutive mTORC1 hyperactivity on cerebellar morphology and cellular organization in a rat model of autism and epilepsy. It describes age-dependent degeneration of Purkinje neurons, with demyelination of central tract as well as activation of microglia, and discusses the implications of these changes for neuro-behavioral phenotypes. The described changes provide new indications for the putative mechanisms underlying cerebellar impairments with their age-related onset, which may contribute to the pathobiology of autism, epilepsy, and related disorders. En ligne : http://dx.doi.org/10.1002/aur.2688 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=473 Recent advances in human stem cell-based modeling of Tuberous Sclerosis Complex / Wardiya AFSHAR SABER in Molecular Autism, 11 (2020)
[article]
Titre : Recent advances in human stem cell-based modeling of Tuberous Sclerosis Complex Type de document : Texte imprimé et/ou numérique Auteurs : Wardiya AFSHAR SABER, Auteur ; Mustafa SAHIN, Auteur Article en page(s) : 16 p. Langues : Anglais (eng) Mots-clés : Astrocytes Autism Brain organoids CRISPR/Cas9 Cortical tuber Human pluripotent stem cells Neurons Purkinje neurons Tuberous sclerosis complex Therapeutics, and Quadrant Biosciences and has served on the Scientific Advisory Board of Sage Therapeutics, Roche, Takeda, and PTEN Research Foundation. Index. décimale : PER Périodiques Résumé : Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by epilepsy, intellectual disability, and benign tumors of the brain, heart, skin, and kidney. Animal models have contributed to our understanding of normal and abnormal human brain development, but the construction of models that accurately recapitulate a human pathology remains challenging. Recent advances in stem cell biology with the derivation of human-induced pluripotent stem cells (hiPSCs) from somatic cells from patients have opened new avenues to the study of TSC. This approach combined with gene-editing tools such as CRISPR/Cas9 offers the advantage of preserving patient-specific genetic background and the ability to generate isogenic controls by correcting a specific mutation. The patient cell line and the isogenic control can be differentiated into the cell type of interest to model various aspects of TSC. In this review, we discuss the remarkable capacity of these cells to be used as a model for TSC in two- and three-dimensional cultures, the potential variability in iPSC models, and highlight differences between findings reported to date. En ligne : http://dx.doi.org/10.1186/s13229-020-0320-2 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=427
in Molecular Autism > 11 (2020) . - 16 p.[article] Recent advances in human stem cell-based modeling of Tuberous Sclerosis Complex [Texte imprimé et/ou numérique] / Wardiya AFSHAR SABER, Auteur ; Mustafa SAHIN, Auteur . - 16 p.
Langues : Anglais (eng)
in Molecular Autism > 11 (2020) . - 16 p.
Mots-clés : Astrocytes Autism Brain organoids CRISPR/Cas9 Cortical tuber Human pluripotent stem cells Neurons Purkinje neurons Tuberous sclerosis complex Therapeutics, and Quadrant Biosciences and has served on the Scientific Advisory Board of Sage Therapeutics, Roche, Takeda, and PTEN Research Foundation. Index. décimale : PER Périodiques Résumé : Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by epilepsy, intellectual disability, and benign tumors of the brain, heart, skin, and kidney. Animal models have contributed to our understanding of normal and abnormal human brain development, but the construction of models that accurately recapitulate a human pathology remains challenging. Recent advances in stem cell biology with the derivation of human-induced pluripotent stem cells (hiPSCs) from somatic cells from patients have opened new avenues to the study of TSC. This approach combined with gene-editing tools such as CRISPR/Cas9 offers the advantage of preserving patient-specific genetic background and the ability to generate isogenic controls by correcting a specific mutation. The patient cell line and the isogenic control can be differentiated into the cell type of interest to model various aspects of TSC. In this review, we discuss the remarkable capacity of these cells to be used as a model for TSC in two- and three-dimensional cultures, the potential variability in iPSC models, and highlight differences between findings reported to date. En ligne : http://dx.doi.org/10.1186/s13229-020-0320-2 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=427