scholarly journals CHARGE syndrome modeling using patient-iPSCs reveals defective migration of neural crest cells harboring CHD7 mutations

eLife ◽  
2017 ◽  
Vol 6 ◽  
Author(s):  
Hironobu Okuno ◽  
Francois Renault Mihara ◽  
Shigeki Ohta ◽  
Kimiko Fukuda ◽  
Kenji Kurosawa ◽  
...  
Keyword(s):  
Neural Crest ◽  
Pluripotent Stem Cells ◽  
Neural Crest Cells ◽  
Charge Syndrome ◽  
In Ovo ◽  
Migratory Activity ◽  
Expression Of Genes ◽  
Induced Pluripotent ◽  
Neural Crest Migration

CHARGE syndrome is caused by heterozygous mutations in the chromatin remodeler, CHD7, and is characterized by a set of malformations that, on clinical grounds, were historically postulated to arise from defects in neural crest formation during embryogenesis. To better delineate neural crest defects in CHARGE syndrome, we generated induced pluripotent stem cells (iPSCs) from two patients with typical syndrome manifestations, and characterized neural crest cells differentiated in vitro from these iPSCs (iPSC-NCCs). We found that expression of genes associated with cell migration was altered in CHARGE iPSC-NCCs compared to control iPSC-NCCs. Consistently, CHARGE iPSC-NCCs showed defective delamination, migration and motility in vitro, and their transplantation in ovo revealed overall defective migratory activity in the chick embryo. These results support the historical inference that CHARGE syndrome patients exhibit defects in neural crest migration, and provide the first successful application of patient-derived iPSCs in modeling craniofacial disorders.

scholarly journals In vivo regeneration of rat laryngeal cartilage with mesenchymal stem cells derived from human induced pluripotent stem cells via neural crest cells

2021 ◽  
Vol 52 ◽  
pp. 102233
Author(s):  
Masayoshi Yoshimatsu ◽  
Hiroe Ohnishi ◽  
Chengzhu Zhao ◽  
Yasuyuki Hayashi ◽  
Fumihiko Kuwata ◽  
...  
Keyword(s):  
Stem Cells ◽  
Mesenchymal Stem Cells ◽  
Neural Crest ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Neural Crest Cells ◽  
Laryngeal Cartilage ◽  
Induced Pluripotent

Evidence for collapsin-1 functioning in the control of neural crest migration in both trunk and hindbrain regions

Development ◽  
1999 ◽  
Vol 126 (10) ◽  
pp. 2181-2189 ◽  
Author(s):  
B.J. Eickholt ◽  
S.L. Mackenzie ◽  
A. Graham ◽  
F.S. Walsh ◽  
P. Doherty
Keyword(s):  
Cell Migration ◽  
Neural Crest ◽  
Neural Crest Cell ◽  
Axon Growth ◽  
Neural Crest Cells ◽  
Neural Crest Cell Migration ◽  
Neural Crest Migration ◽  
Migration Pathways ◽  
Crest Cell

Collapsin-1 belongs to the Semaphorin family of molecules, several members of which have been implicated in the co-ordination of axon growth and guidance. Collapsin-1 can function as a selective chemorepellent for sensory neurons, however, its early expression within the somites and the cranial neural tube (Shepherd, I., Luo, Y., Raper, J. A. and Chang, S. (1996) Dev. Biol. 173, 185–199) suggest that it might contribute to the control of additional developmental processes in the chick. We now report a detailed study on the expression of collapsin-1 as well as on the distribution of collapsin-1-binding sites in regions where neural crest cell migration occurs. collapsin-1 expression is detected in regions bordering neural crest migration pathways in both the trunk and hindbrain regions and a receptor for collapsin-1, neuropilin-1, is expressed by migrating crest cells derived from both regions. When added to crest cells in vitro, a collapsin-1-Fc chimeric protein induces morphological changes similar to those seen in neuronal growth cones. In order to test the function of collapsin-1 on the migration of neural crest cells, an in vitro assay was used in which collapsin-1-Fc was immobilised in alternating stripes consisting of collapsin-Fc/fibronectin versus fibronectin alone. Explanted neural crest cells derived from both trunk and hindbrain regions avoided the collapsin-Fc-containing substratum. These results suggest that collapsin-1 signalling can contribute to the patterning of neural crest cell migration in the developing chick.

scholarly journals Immunological Properties of Neural Crest Cells Derived from Human Induced Pluripotent Stem Cells

2019 ◽  
Vol 28 (1) ◽  
pp. 28-43 ◽  
Author(s):  
Shota Fujii ◽  
Satoru Yoshida ◽  
Emi Inagaki ◽  
Shin Hatou ◽  
Kazuo Tsubota ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Crest ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Neural Crest Cells ◽  
Immunological Properties ◽  
Induced Pluripotent

scholarly journals Neural crest stem cells can be induced in vitro from human-induced pluripotent stem cells using a novel protocol free of feeder cells

2021 ◽  
Vol 16 (3) ◽  
pp. 143-147
Author(s):  
Rei Abe ◽  
Kazuyo Yamauchi ◽  
Kazuki Kuniyoshi ◽  
Takane Suzuki ◽  
Yusuke Matsuura ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Crest ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Feeder Cells ◽  
Neural Crest Stem Cells ◽  
Induced Pluripotent

scholarly journals Robust induction of neural crest cells to derive peripheral sensory neurons from human induced pluripotent stem cells

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Yoshie Umehara ◽  
Sumika Toyama ◽  
Mitsutoshi Tominaga ◽  
Hironori Matsuda ◽  
Nobuaki Takahashi ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Crest ◽  
Induced Pluripotent Stem Cells ◽  
Sensory Neurons ◽  
Pluripotent Stem Cells ◽  
Neural Crest Cells ◽  
Peripheral Sensory Neurons ◽  
Induced Pluripotent ◽  
Peripheral Sensory

scholarly journals MAPRE2 mutations result in altered human cranial neural crest migration, underlying craniofacial malformations in CSC-KT syndrome

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cedric Thues ◽  
Jorge S. Valadas ◽  
Liesbeth Deaulmerie ◽  
Ann Geens ◽  
Amit K. Chouhan ◽  
...  
Keyword(s):  
Neural Crest ◽  
Induced Pluripotent Stem Cell ◽  
Neural Crest Cells ◽  
Branchial Arch ◽  
Cellular Migration ◽  
Cranial Neural Crest ◽  
Craniofacial Malformations ◽  
Neural Crest Migration

AbstractCircumferential skin creases (CSC-KT) is a rare polymalformative syndrome characterised by intellectual disability associated with skin creases on the limbs, and very characteristic craniofacial malformations. Previously, heterozygous and homozygous mutations in MAPRE2 were found to be causal for this disease. MAPRE2 encodes for a member of evolutionary conserved microtubule plus end tracking proteins, the end binding (EB) family. Unlike MAPRE1 and MAPRE3, MAPRE2 is not required for the persistent growth and stabilization of microtubules, but plays a role in other cellular processes such as mitotic progression and regulation of cell adhesion. The mutations identified in MAPRE2 all reside within the calponin homology domain, responsible to track and interact with the plus-end tip of growing microtubules, and previous data showed that altered dosage of MAPRE2 resulted in abnormal branchial arch patterning in zebrafish. In this study, we developed patient derived induced pluripotent stem cell lines for MAPRE2, together with isogenic controls, using CRISPR/Cas9 technology, and differentiated them towards neural crest cells with cranial identity. We show that changes in MAPRE2 lead to alterations in neural crest migration in vitro but also in vivo, following xenotransplantation of neural crest progenitors into developing chicken embryos. In addition, we provide evidence that changes in focal adhesion might underlie the altered cell motility of the MAPRE2 mutant cranial neural crest cells. Our data provide evidence that MAPRE2 is involved in cellular migration of cranial neural crest and offers critical insights into the mechanism underlying the craniofacial dysmorphisms and cleft palate present in CSC-KT patients. This adds the CSC-KT disorder to the growing list of neurocristopathies.

scholarly journals Neural crest cells and their potential therapeutic applications

Morphologia ◽  
2021 ◽  
Vol 15 (3) ◽  
pp. 39-49
Author(s):  
K.M. Shevchenko
Keyword(s):  
Stem Cells ◽  
Embryonic Stem Cells ◽  
Neural Crest ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Embryonic Stem ◽  
Neural Crest Cells ◽  
Red Bone Marrow ◽  
Induced Pluripotent ◽  
Promising Source

Neural crest (NC) is a population of cells, formed at the intersection between non-neural ectoderm and neural tube. Neural crest progenitors are multipotent, have capacity to extensive migration and self-renewal. They can be differentiated into various cells types from craniofacial skeletal tissues to components of peripheral nervous system. Influence of signaling molecules and transcription factors, which are expressed at the different stages regulate development of NC. The regulatory network of genes determines the processes of induction, specification, migration and differentiation of neural crest cells (NCC). The purpose of this article is to compare the characteristics of NCC, obtained from tissues of the embryo, fetus and adult; experimental strategies for obtaining NCC from embryonic stem cells, induced pluripotent stem cells, skin fibroblasts; comparison of the potential of different cell types for therapeutic use in a clinical setting. Embryonic stem NCC are differentiated to the trunk, cranial, cardiac, circumpharyngeal and vagal according to the area of their initial migration. Mature stem NCC can be obtained from the dorsal root ganglia, red bone marrow, hair follicle, skin, intestines, carotid body, heart, cornea, iris, dental pulp, hard palate and oral mucosa. Genetic mutations may lead to failure of regulation of NC development, which leads to many congenital human diseases such as cardiovascular defects, craniofacial abnormalities and intestinal aganglionosis, collectively known as neurocristopathies. The identification and isolation of multipotent stem NCC derived from adult tissues, embryonic stem cells, and induced pluripotent stem cells are promising source for regenerative medicine.

scholarly journals The distribution of tenascin coincides with pathways of neural crest cell migration

Development ◽  
1988 ◽  
Vol 102 (1) ◽  
pp. 237-250 ◽  
Author(s):  
E.J. Mackie ◽  
R.P. Tucker ◽  
W. Halfter ◽  
R. Chiquet-Ehrismann ◽  
H.H. Epperlein
Keyword(s):  
Xenopus Laevis ◽  
Neural Crest ◽  
Neural Crest Cell ◽  
Neural Crest Cells ◽  
Rat Embryos ◽  
The Matrix ◽  
Neural Crest Cell Migration ◽  
Neural Crest Migration ◽  
Migration Pathways

The distribution of the extracellular matrix (ECM) glycoprotein, tenascin, has been compared with that of fibronectin in neural crest migration pathways of Xenopus laevis, quail and rat embryos. In all species studied, the distribution of tenascin, examined by immunohistochemistry, was more closely correlated with pathways of migration than that of fibronectin, which is known to be important for neural crest migration. In Xenopus laevis embryos, anti-tenascin stained the dorsal fin matrix and ECM along the ventral route of migration, but not the ECM found laterally between the ectoderma and somites where neural crest cells do not migrate. In quail embryos, the appearance of tenascin in neural crest pathways was well correlated with the anterior-to-posterior wave of migration. The distribution of tenascin within somites was compared with that of the neural crest marker, HNK-1, in quail embryos. In the dorsal halves of quail somites which contained migrating neural crest cells, the predominant tenascin staining was in the anterior halves of the somites, codistributed with the migrating cells. In rat embryos, tenascin was detectable in the somites only in the anterior halves. Tenascin was not detectable in the matrix of cultured quail neural crest cells, but was in the matrix surrounding somite and notochord cells in vitro. Neural crest cells cultured on a substratum of tenascin did not spread and were rounded. We propose that tenascin is an important factor controlling neural crest morphogenesis, perhaps by modifying the interaction of neural crest cells with fibronectin.

P63Derivation of neural crest cells from human pluripotent stem cells enables modelling of treacher collins syndrome in vitro

2018 ◽  
Vol 114 (suppl_1) ◽  
pp. S16-S16
Author(s):  
F Serrano ◽  
W G Bernard ◽  
A Granata ◽  
D Iyer ◽  
M Kim ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Crest ◽  
Pluripotent Stem Cells ◽  
Neural Crest Cells ◽  
Human Pluripotent Stem Cells ◽  
Treacher Collins Syndrome
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