Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats / Y. WU in Journal of Neurodevelopmental Disorders, 8-1 (December 2016)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 8-1 (December 2016) . - p.23 Titre : Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats Type de document : Texte imprimé et/ou numérique Auteurs : Y. WU, Auteur ; W. ZHONG, Auteur ; N. CUI, Auteur ; C. M. JOHNSON, Auteur ; H. XING, Auteur ; S. ZHANG, Auteur ; C. JIANG, Auteur Article en page(s) : p.23 Langues : Anglais (eng) Mots-clés : Behaviors  Breathing  Locus coeruleus  Mecp2-null rat  Rett syndrome Index. décimale : PER Périodiques Résumé : BACKGROUND: Rett Syndrome (RTT) is a neurodevelopmental disease caused by the disruption of the MECP2 gene. Several mouse models of RTT have been developed with Mecp2 disruptions. Although the mouse models are widely used in RTT research, results obtained need to be validated in other species. Therefore, we performed these studies to characterize phenotypes of a novel Mecp2 (-/Y) rat model and compared them with the Mecp2 (tm1.1Bird) mouse model of RTT. METHODS: RTT-like phenotypes were systematically studied and compared between Mecp2 (-/Y) rats and Mecp2 (-/Y) mice. In-cage conditions of the rats were monitored. Grip strength and spontaneous locomotion were used to evaluate the motor function. Three-chamber test was performed to show autism-type behaviors. Breathing activity was recorded with the plethysmograph. Individual neurons in the locus coeruleus (LC) were studied in the whole-cell current clamp. The lifespan of the rats was determined with their survival time. RESULTS: Mecp2 (-/Y) rats displayed growth retardation, malocclusion, and lack of movements, while hindlimb clasping was not seen. They had weaker forelimb grip strength and a lower rate of locomotion than the WT littermates. Defects in social interaction with other rats were obvious. Breathing frequency variation and apnea in the null rats were significantly higher than in the WT. LC neurons in the null rats showed excessive firing activity. A half of the null rats died in 2 months. Most of the RTT-like symptoms were comparable to those seen in Mecp2 (-/Y) mice, while some appeared more or less severe. The findings that most RTT-like symptoms exist in the rat model with moderate variations and differences from the mouse models support the usefulness of both Mecp2 (-/Y) rodent models. CONCLUSIONS: The novel Mecp2 (-/Y) rat model recapitulated numerous RTT-like symptoms as Mecp2 (-/Y) mouse models did, which makes it a valuable alternative model in the RTT studies when the body size matters. En ligne : http://dx.doi.org/10.1186/s11689-016-9156-7 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=348 [article] Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats [Texte imprimé et/ou numérique] / Y. WU, Auteur ; W. ZHONG, Auteur ; N. CUI, Auteur ; C. M. JOHNSON, Auteur ; H. XING, Auteur ; S. ZHANG, Auteur ; C. JIANG, Auteur . - p.23. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 8-1 (December 2016) . - p.23 Mots-clés : Behaviors  Breathing  Locus coeruleus  Mecp2-null rat  Rett syndrome Index. décimale : PER Périodiques Résumé : BACKGROUND: Rett Syndrome (RTT) is a neurodevelopmental disease caused by the disruption of the MECP2 gene. Several mouse models of RTT have been developed with Mecp2 disruptions. Although the mouse models are widely used in RTT research, results obtained need to be validated in other species. Therefore, we performed these studies to characterize phenotypes of a novel Mecp2 (-/Y) rat model and compared them with the Mecp2 (tm1.1Bird) mouse model of RTT. METHODS: RTT-like phenotypes were systematically studied and compared between Mecp2 (-/Y) rats and Mecp2 (-/Y) mice. In-cage conditions of the rats were monitored. Grip strength and spontaneous locomotion were used to evaluate the motor function. Three-chamber test was performed to show autism-type behaviors. Breathing activity was recorded with the plethysmograph. Individual neurons in the locus coeruleus (LC) were studied in the whole-cell current clamp. The lifespan of the rats was determined with their survival time. RESULTS: Mecp2 (-/Y) rats displayed growth retardation, malocclusion, and lack of movements, while hindlimb clasping was not seen. They had weaker forelimb grip strength and a lower rate of locomotion than the WT littermates. Defects in social interaction with other rats were obvious. Breathing frequency variation and apnea in the null rats were significantly higher than in the WT. LC neurons in the null rats showed excessive firing activity. A half of the null rats died in 2 months. Most of the RTT-like symptoms were comparable to those seen in Mecp2 (-/Y) mice, while some appeared more or less severe. The findings that most RTT-like symptoms exist in the rat model with moderate variations and differences from the mouse models support the usefulness of both Mecp2 (-/Y) rodent models. CONCLUSIONS: The novel Mecp2 (-/Y) rat model recapitulated numerous RTT-like symptoms as Mecp2 (-/Y) mouse models did, which makes it a valuable alternative model in the RTT studies when the body size matters. En ligne : http://dx.doi.org/10.1186/s11689-016-9156-7 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=348

Effects of early-life exposure to THIP on phenotype development in a mouse model of Rett syndrome / W. ZHONG in Journal of Neurodevelopmental Disorders, 8-1 (December 2016)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 8-1 (December 2016) . - p.37 Titre : Effects of early-life exposure to THIP on phenotype development in a mouse model of Rett syndrome Type de document : Texte imprimé et/ou numérique Auteurs : W. ZHONG, Auteur ; C. M. JOHNSON, Auteur ; Y. WU, Auteur ; N. CUI, Auteur ; H. XING, Auteur ; S. ZHANG, Auteur ; C. JIANG, Auteur Article en page(s) : p.37 Langues : Anglais (eng) Mots-clés : Behavior  Gaboxadol  Locus coeruleus  Mecp2  Rett syndrome  Thip Index. décimale : PER Périodiques Résumé : BACKGROUND: Rett syndrome (RTT) is a neurodevelopmental disorder caused mostly by disruptions in the MECP2 gene. MECP2-null mice show imbalances in neuronal excitability and synaptic communications. Several previous studies indicate that augmenting synaptic GABA receptors (GABAARs) can alleviate RTT-like symptoms in mice. In addition to the synaptic GABAARs, there is a group of GABAARs found outside the synaptic cleft with the capability to produce sustained inhibition, which may be potential therapeutic targets for the control of neuronal excitability in RTT. METHODS: Wild-type and MECP2-null mice were randomly divided into four groups, receiving the extrasynaptic GABAAR agonist 4,5,6,7-tetrahydroisoxazolo[5,4-c]pyridin-3-ol hydrochloride (THIP) and vehicle control, respectively. Low-dose THIP was administered to neonatal mice through lactation. RTT-like symptoms including lifespan, breathing, motor function, and social behaviors were studied when mice became mature. Changes in neuronal excitability and norepinephrine biosynthesis enzyme expression were studied in electrophysiology and molecular biology. RESULTS: With no evident sedation and other adverse side effects, early-life exposure to THIP extended the lifespan, alleviated breathing abnormalities, enhanced motor function, and improved social behaviors of MECP2-null mice. Such beneficial effects were associated with stabilization of locus coeruleus neuronal excitability and improvement of norepinephrine biosynthesis enzyme expression. CONCLUSIONS: THIP treatment in early lives might be a therapeutic approach to RTT-like symptoms in MECP2-null mice and perhaps in people with RTT as well. En ligne : http://dx.doi.org/10.1186/s11689-016-9169-2 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=349 [article] Effects of early-life exposure to THIP on phenotype development in a mouse model of Rett syndrome [Texte imprimé et/ou numérique] / W. ZHONG, Auteur ; C. M. JOHNSON, Auteur ; Y. WU, Auteur ; N. CUI, Auteur ; H. XING, Auteur ; S. ZHANG, Auteur ; C. JIANG, Auteur . - p.37. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 8-1 (December 2016) . - p.37 Mots-clés : Behavior  Gaboxadol  Locus coeruleus  Mecp2  Rett syndrome  Thip Index. décimale : PER Périodiques Résumé : BACKGROUND: Rett syndrome (RTT) is a neurodevelopmental disorder caused mostly by disruptions in the MECP2 gene. MECP2-null mice show imbalances in neuronal excitability and synaptic communications. Several previous studies indicate that augmenting synaptic GABA receptors (GABAARs) can alleviate RTT-like symptoms in mice. In addition to the synaptic GABAARs, there is a group of GABAARs found outside the synaptic cleft with the capability to produce sustained inhibition, which may be potential therapeutic targets for the control of neuronal excitability in RTT. METHODS: Wild-type and MECP2-null mice were randomly divided into four groups, receiving the extrasynaptic GABAAR agonist 4,5,6,7-tetrahydroisoxazolo[5,4-c]pyridin-3-ol hydrochloride (THIP) and vehicle control, respectively. Low-dose THIP was administered to neonatal mice through lactation. RTT-like symptoms including lifespan, breathing, motor function, and social behaviors were studied when mice became mature. Changes in neuronal excitability and norepinephrine biosynthesis enzyme expression were studied in electrophysiology and molecular biology. RESULTS: With no evident sedation and other adverse side effects, early-life exposure to THIP extended the lifespan, alleviated breathing abnormalities, enhanced motor function, and improved social behaviors of MECP2-null mice. Such beneficial effects were associated with stabilization of locus coeruleus neuronal excitability and improvement of norepinephrine biosynthesis enzyme expression. CONCLUSIONS: THIP treatment in early lives might be a therapeutic approach to RTT-like symptoms in MECP2-null mice and perhaps in people with RTT as well. En ligne : http://dx.doi.org/10.1186/s11689-016-9169-2 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=349

Individuals with autism spectrum disorder are impaired in absolute but not relative pitch and duration matching in speech and song imitation / L. WANG in Autism Research, 14-11 (November 2021)  

Public ISBD [article]  inAutism Research > 14-11 (November 2021) . - p.2355-2372 Titre : Individuals with autism spectrum disorder are impaired in absolute but not relative pitch and duration matching in speech and song imitation Type de document : Texte imprimé et/ou numérique Auteurs : L. WANG, Auteur ; P. Q. PFORDRESHER, Auteur ; C. JIANG, Auteur ; F. LIU, Auteur Article en page(s) : p.2355-2372 Langues : Anglais (eng) Mots-clés : Adult  Autism Spectrum Disorder/complications  Child  Humans  Imitative Behavior  Singing  Speech  Voice  Asd  duration  pitch  song  speech  vocal imitation Index. décimale : PER Périodiques Résumé : Individuals with autism spectrum disorder (ASD) often exhibit atypical imitation. However, few studies have identified clear quantitative characteristics of vocal imitation in ASD. This study investigated imitation of speech and song in English-speaking individuals with and without ASD and its modulation by age. Participants consisted of 25 autistic children and 19 autistic adults, who were compared to 25 children and 19 adults with typical development matched on age, gender, musical training, and cognitive abilities. The task required participants to imitate speech and song stimuli with varying pitch and duration patterns. Acoustic analyses of the imitation performance suggested that individuals with ASD were worse than controls on absolute pitch and duration matching for both speech and song imitation, although they performed as well as controls on relative pitch and duration matching. Furthermore, the two groups produced similar numbers of pitch contour, pitch interval-, and time errors. Across both groups, sung pitch was imitated more accurately than spoken pitch, whereas spoken duration was imitated more accurately than sung duration. Children imitated spoken pitch more accurately than adults when it came to speech stimuli, whereas age showed no significant relationship to song imitation. These results reveal a vocal imitation deficit across speech and music domains in ASD that is specific to absolute pitch and duration matching. This finding provides evidence for shared mechanisms between speech and song imitation, which involves independent implementation of relative versus absolute features. LAY SUMMARY: Individuals with autism spectrum disorder (ASD) often exhibit atypical imitation of actions and gestures. Characteristics of vocal imitation in ASD remain unclear. By comparing speech and song imitation, this study shows that individuals with ASD have a vocal imitative deficit that is specific to absolute pitch and duration matching, while performing as well as controls on relative pitch and duration matching, across speech and music domains. En ligne : http://dx.doi.org/10.1002/aur.2569 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=450 [article] Individuals with autism spectrum disorder are impaired in absolute but not relative pitch and duration matching in speech and song imitation [Texte imprimé et/ou numérique] / L. WANG, Auteur ; P. Q. PFORDRESHER, Auteur ; C. JIANG, Auteur ; F. LIU, Auteur . - p.2355-2372. Langues : Anglais (eng) in Autism Research > 14-11 (November 2021) . - p.2355-2372 Mots-clés : Adult  Autism Spectrum Disorder/complications  Child  Humans  Imitative Behavior  Singing  Speech  Voice  Asd  duration  pitch  song  speech  vocal imitation Index. décimale : PER Périodiques Résumé : Individuals with autism spectrum disorder (ASD) often exhibit atypical imitation. However, few studies have identified clear quantitative characteristics of vocal imitation in ASD. This study investigated imitation of speech and song in English-speaking individuals with and without ASD and its modulation by age. Participants consisted of 25 autistic children and 19 autistic adults, who were compared to 25 children and 19 adults with typical development matched on age, gender, musical training, and cognitive abilities. The task required participants to imitate speech and song stimuli with varying pitch and duration patterns. Acoustic analyses of the imitation performance suggested that individuals with ASD were worse than controls on absolute pitch and duration matching for both speech and song imitation, although they performed as well as controls on relative pitch and duration matching. Furthermore, the two groups produced similar numbers of pitch contour, pitch interval-, and time errors. Across both groups, sung pitch was imitated more accurately than spoken pitch, whereas spoken duration was imitated more accurately than sung duration. Children imitated spoken pitch more accurately than adults when it came to speech stimuli, whereas age showed no significant relationship to song imitation. These results reveal a vocal imitation deficit across speech and music domains in ASD that is specific to absolute pitch and duration matching. This finding provides evidence for shared mechanisms between speech and song imitation, which involves independent implementation of relative versus absolute features. LAY SUMMARY: Individuals with autism spectrum disorder (ASD) often exhibit atypical imitation of actions and gestures. Characteristics of vocal imitation in ASD remain unclear. By comparing speech and song imitation, this study shows that individuals with ASD have a vocal imitative deficit that is specific to absolute pitch and duration matching, while performing as well as controls on relative pitch and duration matching, across speech and music domains. En ligne : http://dx.doi.org/10.1002/aur.2569 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=450

The autistic brain can process local but not global emotion regularities in facial and musical sequences / J. XU in Autism Research, 15-2 (February 2022)  

Public ISBD [article]  inAutism Research > 15-2 (February 2022) . - p.222-240 Titre : The autistic brain can process local but not global emotion regularities in facial and musical sequences Type de document : Texte imprimé et/ou numérique Auteurs : J. XU, Auteur ; L. ZHOU, Auteur ; F. LIU, Auteur ; C. XUE, Auteur ; J. JIANG, Auteur ; C. JIANG, Auteur Article en page(s) : p.222-240 Langues : Anglais (eng) Mots-clés : autism spectrum disorder  facial emotion  global deficit  musical emotion  regularity Index. décimale : PER Périodiques Résumé : Whether autism spectrum disorder (ASD) is associated with a global processing deficit remains controversial. Global integration requires extraction of regularity across various timescales, yet little is known about how individuals with ASD process regularity at local (short timescale) versus global (long timescale) levels. To this end, we used event-related potentials to investigate whether individuals with ASD would show different neural responses to local (within trial) versus global (across trials) emotion regularities extracted from sequential facial expressions; and if so, whether this visual abnormality would generalize to the music (auditory) domain. Twenty individuals with ASD and 21 age- and IQ-matched individuals with typical development participated in this study. At an early processing stage, ASD participants exhibited preserved neural responses to violations of local emotion regularity for both faces and music. At a later stage, however, there was an absence of neural responses in ASD to violations of global emotion regularity for both faces and music. These findings suggest that the autistic brain responses to emotion regularity are modulated by the timescale of sequential stimuli, and provide insight into the neural mechanisms underlying emotional processing in ASD. En ligne : http://dx.doi.org/10.1002/aur.2635 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=450 [article] The autistic brain can process local but not global emotion regularities in facial and musical sequences [Texte imprimé et/ou numérique] / J. XU, Auteur ; L. ZHOU, Auteur ; F. LIU, Auteur ; C. XUE, Auteur ; J. JIANG, Auteur ; C. JIANG, Auteur . - p.222-240. Langues : Anglais (eng) in Autism Research > 15-2 (February 2022) . - p.222-240 Mots-clés : autism spectrum disorder  facial emotion  global deficit  musical emotion  regularity Index. décimale : PER Périodiques Résumé : Whether autism spectrum disorder (ASD) is associated with a global processing deficit remains controversial. Global integration requires extraction of regularity across various timescales, yet little is known about how individuals with ASD process regularity at local (short timescale) versus global (long timescale) levels. To this end, we used event-related potentials to investigate whether individuals with ASD would show different neural responses to local (within trial) versus global (across trials) emotion regularities extracted from sequential facial expressions; and if so, whether this visual abnormality would generalize to the music (auditory) domain. Twenty individuals with ASD and 21 age- and IQ-matched individuals with typical development participated in this study. At an early processing stage, ASD participants exhibited preserved neural responses to violations of local emotion regularity for both faces and music. At a later stage, however, there was an absence of neural responses in ASD to violations of global emotion regularity for both faces and music. These findings suggest that the autistic brain responses to emotion regularity are modulated by the timescale of sequential stimuli, and provide insight into the neural mechanisms underlying emotional processing in ASD. En ligne : http://dx.doi.org/10.1002/aur.2635 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=450

---

1 (1 - 4 / 4)