Developmental Neurobiology最新文献_第5页

Characterization of neuropathology in ovine CLN5 and CLN6 neuronal ceroid lipofuscinoses (Batten disease) 绵羊CLN5和CLN6神经元样脂褐变(巴滕病)的神经病理学特征

IF 3 4区医学

Developmental Neurobiology Pub Date : 2023-05-28 DOI: 10.1002/dneu.22918

Nadia L. Mitchell, Katharina N. Russell, Graham K. Barrell, Imke Tammen, David N. Palmer

{"title":"Characterization of neuropathology in ovine CLN5 and CLN6 neuronal ceroid lipofuscinoses (Batten disease)","authors":"Nadia L. Mitchell, Katharina N. Russell, Graham K. Barrell, Imke Tammen, David N. Palmer","doi":"10.1002/dneu.22918","DOIUrl":"10.1002/dneu.22918","url":null,"abstract":"Sheep with naturally occurring CLN5 and CLN6 forms of neuronal ceroid lipofuscinoses (Batten disease) share the key clinical features of the human disease and represent an ideal model system in which the clinical efficacy of gene therapies is developed and test. However, it was first important to characterize the neuropathological changes that occur with disease progression in affected sheep. This study compared neurodegeneration, neuroinflammation, and lysosomal storage accumulation in CLN5 affected Borderdale, CLN6 affected South Hampshire, and Merino sheep brains from birth to end-stage disease at ≤24 months of age. Despite very different gene products, mutations, and subcellular localizations, the pathogenic cascade was remarkably similar for all three disease models. Glial activation was present at birth in affected sheep and preceded neuronal loss, with both spreading from the visual and parieto-occipital cortices most prominently associated with clinical symptoms to the entire cortical mantle by end-stage disease. In contrast, the subcortical regions were less involved, yet lysosomal storage followed a near-linear increase across the diseased sheep brain with age. Correlation of these neuropathological changes with published clinical data identified three potential therapeutic windows in affected sheep—presymptomatic (3 months), early symptomatic (6 months), and a later symptomatic disease stage (9 months of age)—beyond which the extensive depletion of neurons was likely to diminish any chance of therapeutic benefit. This comprehensive natural history of the neuropathological changes in ovine CLN5 and CLN6 disease will be integral in determining what impact treatment has at each of these disease stages.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 5-6","pages":"127-142"},"PeriodicalIF":3.0,"publicationDate":"2023-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/dneu.22918","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10234297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 3

Ccdc25 regulates neurogenesis during the brain development Ccdc25调节大脑发育过程中的神经发生

IF 3 4区医学

Developmental Neurobiology Pub Date : 2023-04-24 DOI: 10.1002/dneu.22911

Chong Wang, Jie Qin, Jianwei Jiao, Fen Ji

{"title":"Ccdc25 regulates neurogenesis during the brain development","authors":"Chong Wang, Jie Qin, Jianwei Jiao, Fen Ji","doi":"10.1002/dneu.22911","DOIUrl":"10.1002/dneu.22911","url":null,"abstract":"During brain development, the proliferation and differentiation of neural stem cells (NSCs) are precisely regulated. Defects in embryonic brain development can lead to serious developmental disorders. The cerebral cortex is the most evolved and complicated structure in the mammalian brain. The process of neuronal production, also known as neurogenesis, plays crucial roles in cerebral development and can affect the function of the neocortex. Ccdc25 is a newly discovered molecule. It has been proved that it can play an important role in tumor. However, its function in neural systems is unclear. In this study, we find that in early embryonic development, Ccdc25 can express in the brain. Suppression of the Ccdc25 mediated by shRNAs causes the increase of the Ki67- or BrdU-positive NSCs proliferation and inhibits the premature terminal mitosis and neuronal differentiation. Simultaneously, overexpression of Ccdc2525 inhibits the proliferation and promotes the differentiation of NSCs. Knockdown of Ccdc25 also affects neuronal maturation, the number of branches of neurons cultured in vitro decreased, and the number of axons became shorter. We also examined the expression profile of NSCs when Ccdc25 was knocked down by RNA sequencing technique. We found that Ccdc25 regulates the development of NSCs through Egr1. Egr1 knockdown can result in a phenotype similar to Ccdc25, while the overexpression of Egr1 can also rescue the phenotype of Ccdc25 knockdown. In conclusion, Ccdc25 can affect the proliferation and differentiation of NSCs and the maturation of neuron.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 3-4","pages":"91-103"},"PeriodicalIF":3.0,"publicationDate":"2023-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9627822","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Ten-m4 plays a unique role in the establishment of binocular visual circuits 10 -m4在双目视觉回路的建立中起着独特的作用

IF 3 4区医学

Developmental Neurobiology Pub Date : 2023-04-24 DOI: 10.1002/dneu.22912

Timothy R. Young, Dylan Black, Hannan Mansuri, Toshitaka Oohashi, Xiao-Hong Zhou, Atomu Sawatari, Catherine A. Leamey

{"title":"Ten-m4 plays a unique role in the establishment of binocular visual circuits","authors":"Timothy R. Young, Dylan Black, Hannan Mansuri, Toshitaka Oohashi, Xiao-Hong Zhou, Atomu Sawatari, Catherine A. Leamey","doi":"10.1002/dneu.22912","DOIUrl":"10.1002/dneu.22912","url":null,"abstract":"The patterning of binocular vision requires distinct molecular pathways for inputs arising from each side of the nervous system. Recent studies have demonstrated important roles for members of the Ten-m/Odz/teneurin family in the development of ipsilateral retinal projections. Here, we further highlight the significance of this gene family in visual development by identifying a role for Ten-m4 during the formation of the ipsilateral projection in the mouse. Ten-m4 was found to be expressed in the retina, dorsal lateral geniculate nucleus (dLGN), superior colliculus (SC), and primary visual cortex (V1) during development. Anterograde and retrograde tracing experiments in Ten-m4 knockout (KO) mice revealed a specific increase in ipsilateral retinal ganglion cells projecting to dLGN and SC. This increase was most prominent in regions corresponding to temporal retina. Consistent with this, EphB1 expression in the retina around the time of decussation was enhanced in this temporal region for KO mice, suggesting that the increased size of the ipsilateral population arises due to an increased number of retinal ganglion cells remaining ipsilaterally at the optic chiasm due to EphB1-mediated repulsion. The ectopic ipsilaterally targeted retinal ganglion cell projection observed in Ten-m4 KOs was associated with changes in response to ethologically relevant visual stimuli. Together, these data demonstrate a requirement for Ten-m4 in the establishment of ipsilateral projections from the retina, which likely acts in combination with other Ten-m members (Ten-m2 and Ten-m3) to promote the formation of functional binocular circuits.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 3-4","pages":"104-124"},"PeriodicalIF":3.0,"publicationDate":"2023-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/dneu.22912","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9627827","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Advances in in utero electroporation 子宫内电穿孔研究进展

IF 3 4区医学

Developmental Neurobiology Pub Date : 2023-03-02 DOI: 10.1002/dneu.22910

Claire M. Kittock, Louis-Jan Pilaz

引用次数: 3

Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response 斑马鱼线粒体Chchd10或Chchd2的缺失会导致als样表型和独立于线粒体综合应激反应的复合物I缺陷

IF 3 4区医学

Developmental Neurobiology Pub Date : 2023-02-16 DOI: 10.1002/dneu.22909

Virginie Petel Légaré, Christian J. Rampal, Tyler J. N. Gurberg, Mari J. Aaltonen, Alexandre Janer, Lorne Zinman, Eric A. Shoubridge, Gary A. B. Armstrong

{"title":"Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response","authors":"Virginie Petel Légaré, Christian J. Rampal, Tyler J. N. Gurberg, Mari J. Aaltonen, Alexandre Janer, Lorne Zinman, Eric A. Shoubridge, Gary A. B. Armstrong","doi":"10.1002/dneu.22909","DOIUrl":"10.1002/dneu.22909","url":null,"abstract":"Mutations in CHCHD10 and CHCHD2, encoding two paralogous mitochondrial proteins, have been identified in cases of amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Parkinson's disease. Their role in disease is unclear, though both have been linked to mitochondrial respiration and mitochondrial stress responses. Here, we investigated the biological roles of these proteins during vertebrate development using knockout (KO) models in zebrafish. We demonstrate that loss of either or both proteins leads to motor impairment, reduced survival and compromised neuromuscular junction integrity in larval zebrafish. Compensation by Chchd10 was observed in the chchd2−/− model, but not by Chchd2 in the chchd10−/− model. The assembly of mitochondrial respiratory chain Complex I was impaired in chchd10−/− and chchd2−/− zebrafish larvae, but unexpectedly not in a double chchd10−/− and chchd2−/− model, suggesting that reduced mitochondrial Complex I cannot be solely responsible for the observed phenotypes, which are generally more severe in the double KO. We observed transcriptional activation markers of the mitochondrial integrated stress response (mt-ISR) in the double chchd10−/− and chchd2−/− KO model, suggesting that this pathway is involved in the restoration of Complex I assembly in our double KO model. The data presented here demonstrates that the Complex I assembly defect in our single KO models arises independently of the mt-ISR. Furthermore, this study provides evidence that both proteins are required for normal vertebrate development.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 1-2","pages":"54-69"},"PeriodicalIF":3.0,"publicationDate":"2023-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/dneu.22909","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9292321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Cover Image, Volume 82, Issue 7–8 封面图片，第82卷，第7-8期

IF 3 4区医学

Developmental Neurobiology Pub Date : 2022-11-22 DOI: 10.1002/dneu.22908

引用次数: 0

Excitatory and inhibitory neuron imbalance in the intrauterine growth restricted fetal guinea pig brain: Relevance to the developmental origins of schizophrenia and autism 宫内生长受限的豚鼠胎儿脑中的兴奋性和抑制性神经元失衡:与精神分裂症和自闭症的发育起源相关

IF 3 4区医学

Developmental Neurobiology Pub Date : 2022-11-14 DOI: 10.1002/dneu.22907

Angela Cumberland, Nadia Hale, Aminath Azhan, Courtney P. Gilchrist, Ginevra Chincarini, Mary Tolcos

{"title":"Excitatory and inhibitory neuron imbalance in the intrauterine growth restricted fetal guinea pig brain: Relevance to the developmental origins of schizophrenia and autism","authors":"Angela Cumberland, Nadia Hale, Aminath Azhan, Courtney P. Gilchrist, Ginevra Chincarini, Mary Tolcos","doi":"10.1002/dneu.22907","DOIUrl":"10.1002/dneu.22907","url":null,"abstract":"Neurodevelopmental disorders such as schizophrenia and autism are thought to involve an imbalance of excitatory and inhibitory signaling in the brain. Intrauterine growth restriction (IUGR) is a risk factor for these disorders, with IUGR onset occurring during critical periods of neurodevelopment. The aim of this study was to determine the impact of IUGR on excitatory and inhibitory neurons of the fetal neocortex and hippocampus. Fetal brains (n = 2) were first collected from an unoperated pregnant guinea pig at mid-gestation (32 days of gestation [dg]; term ∼67 dg) to visualize excitatory (Ctip2) and inhibitory (calretinin [CR] and somatostatin [SST]) neurons via immunohistochemistry. Chronic placental insufficiency (CPI) was then induced via radial artery ablation at 30 dg in another cohort of pregnant guinea pigs (n = 8) to generate IUGR fetuses (52 dg; n = 8); control fetuses (52 dg; n = 7) were from sham surgeries with no radial artery ablation. At 32 dg, Ctip2- and CR-immunoreactive (IR) cells had populated the cerebral cortex, whereas SST-IR cells had not, suggesting these neurons were yet to complete migration. At 52 dg, in IUGR versus control fetuses, there was a reduction in SST-IR cell density in the cerebral cortex (p = .0175) and hilus of the dentate gyrus (p = .0035) but not the striatum (p > .05). There was no difference between groups in the density of Ctip2-IR (cortex) or CR-IR (cortex, hippocampus) neurons (p > 0.05). Thus, we propose that an imbalance in inhibitory (SST-IR) and excitatory (Ctip2-IR) neurons in the IUGR fetal guinea pig brain could lead to excitatory/inhibitory dysfunction commonly seen in neurodevelopmental disorders such as autism and schizophrenia.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 1-2","pages":"40-53"},"PeriodicalIF":3.0,"publicationDate":"2022-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/dneu.22907","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9344297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

microRNA-124 regulates Notch and NeuroD1 to mediate transition states of neuronal development microRNA-124调节Notch和NeuroD1介导神经元发育的过渡状态

IF 3 4区医学

Developmental Neurobiology Pub Date : 2022-11-07 DOI: 10.1002/dneu.22902

Kalin D. Konrad, Jia L. Song

{"title":"microRNA-124 regulates Notch and NeuroD1 to mediate transition states of neuronal development","authors":"Kalin D. Konrad, Jia L. Song","doi":"10.1002/dneu.22902","DOIUrl":"10.1002/dneu.22902","url":null,"abstract":"MicroRNAs regulate gene expression by destabilizing target mRNA and/or inhibiting translation in animal cells. The ability to mechanistically dissect miR-124′s function during specification, differentiation, and maturation of neurons during development within a single system has not been accomplished. Using the sea urchin embryo, we take advantage of the manipulability of the embryo and its well-documented gene regulatory networks (GRNs). We incorporated NeuroD1 as part of the sea urchin neuronal GRN and determined that miR-124 inhibition resulted in aberrant gut contractions, swimming velocity, and neuronal development. Inhibition of miR-124 resulted in an increased number of cells expressing transcription factors (TFs) associated with progenitor neurons and a concurrent decrease of mature and functional neurons. Results revealed that in the early blastula/gastrula stages, miR-124 regulates undefined factors during neuronal specification and differentiation. In the late gastrula/larval stages, miR-124 regulates Notch and NeuroD1 during the transition between neuronal differentiation and maturation. Overall, we have improved the neuronal GRN and identified miR-124 to play a prolific role in regulating various transitions of neuronal development.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 1-2","pages":"3-27"},"PeriodicalIF":3.0,"publicationDate":"2022-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10440801/pdf/nihms-1909457.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10043228","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

Family income buffers the relationship between childhood adverse experiences and putamen volume 家庭收入缓冲了童年不良经历与壳核体积之间的关系

IF 3 4区医学

Developmental Neurobiology Pub Date : 2022-10-31 DOI: 10.1002/dneu.22906

Max P. Herzberg, Laura Hennefield, Katherine R. Luking, Ashley F. P. Sanders, Alecia C. Vogel, Sridhar Kandala, Rebecca Tillman, Joan Luby, Deanna M. Barch

{"title":"Family income buffers the relationship between childhood adverse experiences and putamen volume","authors":"Max P. Herzberg, Laura Hennefield, Katherine R. Luking, Ashley F. P. Sanders, Alecia C. Vogel, Sridhar Kandala, Rebecca Tillman, Joan Luby, Deanna M. Barch","doi":"10.1002/dneu.22906","DOIUrl":"10.1002/dneu.22906","url":null,"abstract":"Adverse experiences and family income in childhood have been associated with altered brain development. While there is a large body of research examining these associations, it has primarily used cross-sectional data sources and studied adverse experiences and family income in isolation. However, it is possible that low family income and adverse experiences represent dissociable and potentially interacting profiles of risk. To address this gap in the literature, we examined brain structure as a function of adverse experiences in childhood and family income in 158 youths with up to five waves of MRI data. Specifically, we assessed the interactive effect of these two risk factors on six regions of interest: hippocampus, putamen, amygdala, nucleus accumbens, caudate, and thalamus. Adverse experiences and family income interacted to predict putamen volume (B = 0.086, p = 0.011) but only in participants with family income one standard deviation below the mean (slope estimate = −0.11, p = 0.03). These results suggest that adverse experiences in childhood result in distinct patterns of brain development across the socioeconomic gradient. Given previous findings implicating the role of the putamen in psychopathology-related behaviors, these results emphasize the importance of considering life events and socioeconomic context when evaluating markers of risk. Future research should include interactive effects of environmental exposures and family income to better characterize risk for psychopathology in diverse samples.","PeriodicalId":11300,"journal":{"name":"Developmental Neurobiology","volume":"83 1-2","pages":"28-39"},"PeriodicalIF":3.0,"publicationDate":"2022-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/dneu.22906","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9291288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Microglia in motor neuron disease: Signaling evidence from last 10 years 运动神经元疾病中的小胶质细胞:近10年的信号证据

IF 3 4区医学

Developmental Neurobiology Pub Date : 2022-10-29 DOI: 10.1002/dneu.22905

Min-Jia Wang, Lu Kang, Yao-Zheng Wang, Bi-Ru Yang, Chun Zhang, Yu-Feng Lu, Liang Kang

引用次数: 7