Cellular and Axonal Constituents of Neocortical Molecular Layer Heterotopia

Ramos, Raddy L.; Siu, Nga Yan; Brunken, William J.; Yee, Kathleen T.; Gabel, Lisa A.; Dine, Sarah E. Van; Hoplight, Blair J.

doi:10.1159/000365100

Cited by 11 publications

(16 citation statements)

References 73 publications

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“…The prevalence of MLH in C57BL/6J and C57BL/10J strains is well established [25–27], but only a small number of brains from a few other inbred mouse strains have been examined [25]. Consequently, we used well-established histological methods [25–27] to examine brains from numerous inbred mice including: 129S6/SvEvTac (n=20), 129S1/SvImJ (n=17), A/J (n=17), BALB/cJ (n=16), DBA/2J (n=20), FVB/NJ (n=18) strains.…”

Section: Resultsmentioning

confidence: 99%

“…Consequently, we used well-established histological methods [25–27] to examine brains from numerous inbred mice including: 129S6/SvEvTac (n=20), 129S1/SvImJ (n=17), A/J (n=17), BALB/cJ (n=16), DBA/2J (n=20), FVB/NJ (n=18) strains. We used methods previously described [25,27,39,41,42] to examine high-resolution digital histological material from the Mouse Strains dataset from the ABA, including 129S1/SvImJ (n=117 cases), C57BL/6J (n=314), Cast/EiJ (n=117 cases), DBA/2J (n=117 cases), PWD/PhJ (n=93 cases), SPRET/EiJ (n=117 cases), WSB/EiJ (n=93 cases) strains. Remarkably, we did not observe MLH in any inbred strain except C57BL/6J brains in the ABA Mouse Strains dataset.…”

Section: Resultsmentioning

confidence: 99%

“…We also used digital histological data from the Allen Brain Atlas (ABA; www.brain-map.org) and Mouse Brain Architecture Project (MBAP; http://mouse.brainarchitecture.org) to document heterotopia as previously described [25,27,39,41,42]. Data from the ABA Mouse Strains dataset contains high-resolution sagittal and coronal sections from the following strains (The Jackson Laboratory): 129S1/SvlmJ, C57BL/6J, Cast/EiJ, DBA/2J, PWD/PhJ, SPRET/EiJ, WSB/EiJ.…”

Section: Methodsmentioning

confidence: 99%

“…MLH are present in several knockout (KO) lines [13–22] and inbred mouse strains [23,24], including C57BL/6 and C57BL/10 mice [25–27]. The cytoarchitecture of MLH in mice is indistinguishable from human heterotopia, suggesting similar causal mechanisms.…”

Section: Introductionmentioning

confidence: 99%

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Divergence and inheritance of neocortical heterotopia in inbred and genetically-engineered mice

Toia

Cuoco

Esposito

et al. 2017

Neuroscience Letters

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Cortical function emerges from the intrinsic properties of neocortical neurons and their synaptic connections within and across lamina. Neurodevelopmental disorders affecting migration and lamination of the neocortex result in cognitive delay/disability and epilepsy. Molecular layer heterotopia (MLH), a dysplasia characterized by over-migration of neurons into layer I, are associated with cognitive deficits and neuronal hyperexcitability in humans and mice. The breadth of different inbred mouse strains that exhibit MLH and inheritance patterns of heterotopia remain unknown. A neuroanatomical survey of numerous different inbred mouse strains, 2 first filial generation (F1) hybrids, and one consomic strain (C57BL/6J-Chr 1A/J/NaJ) revealed MLH only in C57BL/6 mice and the consomic strain. Heterotopia were observed in numerous genetically-engineered mouse lines on a congenic C57BL/6 background. These data indicate that heterotopia formation is a weakly penetrant trait requiring homozygosity of one or more C57BL/6 alleles outside of chromosome 1. These data are relevant toward understanding neocortical development and disorders affecting neocortical lamination.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Divergence and inheritance of neocortical heterotopia in inbred and genetically-engineered mice

Toia

Cuoco

Esposito

et al. 2017

Neuroscience Letters

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“…We have extensively described our approach of using digital histological data found in the Allen Brain Atlas (ABA; brain-map.org) as a tool to identify the ontogeny, cell types, axonal constituents, and gene expression profiles of cerebellar [19–22] and neocortical heterotopia [32, 33]. In the present report, we specifically used data from the Mouse Connectivity database, which includes histology from neuronal tracing experiments performed in C57BL/6J mice as well as in diverse Cre- driver lines [34–36].…”

Section: Methodsmentioning

confidence: 99%

Malformation of the Posterior Cerebellar Vermis Is a Common Neuroanatomical Phenotype of Genetically Engineered Mice on the C57BL/6 Background

Cuoco

Esposito²,

Moriarty³

et al. 2017

Cerebellum

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C57BL/6 mice exhibit spontaneous cerebellar malformations consisting of heterotopic neurons and glia in the molecular layer of the posterior vermis, indicative of neuronal migration defect during cerebellar development. Recognizing that many genetically engineered (GE) mouse lines are produced from C57BL/6 ES cells or backcrossed to this strain, we performed histological analyses and found that cerebellar heterotopia were a common feature present in the majority of GE lines on this background. Furthermore, we identify GE mouse lines that will be valuable in the study of cerebellar malformations including diverse driver, reporter, and optogenetic lines. Finally, we discuss the implications that these data have on the use of C57BL/6 mice and GE mice on this background in studies of cerebellar development or as models of disease.

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Prolidase enzyme is required for extracellular matrix integrity and impacts on postnatal cerebellar cortex development

Insolia

Priori

Gasperini

et al. 2019

J of Comparative Neurology

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The extracellular matrix is essential for brain development, lamination, and synaptogenesis. In particular, the basement membrane below the pial meninx (pBM) is required for correct cortical development. The last step in the catabolism of the most abundant protein in pBM, collagen Type IV, requires prolidase, an exopeptidase cleaving the imidodipeptides containing pro or hyp at the C‐terminal end. Mutations impairing prolidase activity lead in humans to the rare disease prolidase deficiency characterized by severe skin ulcers and mental impairment. Thus, the dark‐like (dal) mouse, in which the prolidase is knocked‐out, was used to investigate whether the deficiency of prolidase affects the neuronal maturation during development of a brain cortex area. Focusing on the cerebellar cortex, thinner collagen fibers and disorganized pBM were found. Aberrant cortical granule cell proliferation and migration occurred, associated to defects in brain lamination, and in particular in maturation of Purkinje neurons and formation of synaptic contacts. This study deeply elucidates a link between prolidase activity and neuronal maturation shedding new light on the molecular basis of functional aspects in the prolidase deficiency.

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Cellular and Axonal Constituents of Neocortical Molecular Layer Heterotopia

Cited by 11 publications

References 73 publications

Divergence and inheritance of neocortical heterotopia in inbred and genetically-engineered mice

Divergence and inheritance of neocortical heterotopia in inbred and genetically-engineered mice

Malformation of the Posterior Cerebellar Vermis Is a Common Neuroanatomical Phenotype of Genetically Engineered Mice on the C57BL/6 Background

Prolidase enzyme is required for extracellular matrix integrity and impacts on postnatal cerebellar cortex development

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