Reprogramming Captures the Genetic and Tumorigenic Properties of Neurofibromatosis Type 1 Plexiform Neurofibromas

Carrió, Meritxell; Mazuelas, Helena; Richaud‐Patin, Yvonne; Gel, Bernat; Terribas, Ernest; Rosas, Inma; Jiménez‐Delgado, Senda; Biayna, Josep; Vendredy, Leen; Blanco, Ignacio; Castellanos, Elisabeth; Lázaro, Conxi; Serra, Eduard

doi:10.1016/j.stemcr.2019.02.001

Cited by 8 publications

(25 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… NF1(−/−) hiPSC-Scwann cells exhibited a continuous high proliferation rate and poor myelination ability. [ 33 ] Disease modeling for CHARGE syndrome hiPSCs are differentiated into P75/HNK1 (+/+) NCCs using the following two different methods.1)hiPSCs are neurally differentiated via dual SMAD inhibition. 2)Neurospheres are prepared from hiPSCs and spontaneously attach to the petri-dish after day 6–8.…”

Section: Disease Modeling Of Neurocristopathies Using Induced Pluripotent Stem Cells (Ipscs)mentioning

confidence: 99%

“…generated PNF-derived NF1 (−/−) iPSCs and showed that their genomic status enabled their differentiation toward neural crest stem cells and then to stem cells. iPSC-derived NF1 (−/−) stem cells exhibited a high proliferation rate, a poor capacity for myelination, and a tendency to form 3D spheres [ 33 ].…”

Section: Disease Modeling Of Neurocristopathies Using Induced Pluripotent Stem Cells (Ipscs)mentioning

confidence: 99%

See 1 more Smart Citation

Modeling human congenital disorders with neural crest developmental defects using patient-derived induced pluripotent stem cells

Okuno

2021

Regenerative Therapy

View full text Add to dashboard Cite

The neural crest is said to be the fourth germ layer in addition to the ectoderm, mesoderm and endoderm because of its ability to differentiate into a variety of cells that contribute to the various tissues of the vertebrate body. Neural crest cells (NCCs) can be divided into three functional groups: cranial NCCs, cardiac NCCs and trunk NCCs. Defects related to NCCs can contribute to a broad spectrum of syndromes known as neurocristopathies. Studies on the neural crest have been carried out using animal models such as Xenopus , chicks, and mice. However, the precise control of human NCC development has not been elucidated in detail due to species differences. Using induced pluripotent stem cell (iPSC) technology, we developed an in vitro disease model of neurocristopathy by inducing the differentiation of patient-derived iPSCs into NCCs and/or neural crest derivatives. It is now possible to address complicated questions regarding the pathogenetic mechanisms of neurocristopathies by characterizing cellular biological features and transcriptomes and by transplanting patient-derived NCCs in vivo . Here, we provide some examples that elucidate the pathophysiology of neurocristopathies using disease modeling via iPSCs.

show abstract

Section: Disease Modeling Of Neurocristopathies Using Induced Pluripotent Stem Cells (Ipscs)mentioning

confidence: 99%

Section: Disease Modeling Of Neurocristopathies Using Induced Pluripotent Stem Cells (Ipscs)mentioning

confidence: 99%

Modeling human congenital disorders with neural crest developmental defects using patient-derived induced pluripotent stem cells

Okuno

2021

Regenerative Therapy

View full text Add to dashboard Cite

show abstract

“…They also tested different drugs and observed resistance to growth inhibition in cells cultured in 3D. Other groups have demonstrated that induced pluripotent stem cell-(iPSC-) derived NF1 Schwann cells formed 3D spheroid-like structures in culture and expressed specific pNF markers [173]. Indeed, these 3D models are particularly interesting and ben-eficial for the NF1 field, but they are not necessarily suitable for long-term studies.…”

Section: Cutaneous Neurofibromasmentioning

confidence: 99%

Human Organ-Specific 3D Cancer Models Produced by the Stromal Self-Assembly Method of Tissue Engineering for the Study of Solid Tumors

Roy

Magne

Vaillancourt-Audet

et al. 2020

BioMed Research International

View full text Add to dashboard Cite

Cancer research has considerably progressed with the improvement of in vitro study models, helping to understand the key role of the tumor microenvironment in cancer development and progression. Over the last few years, complex 3D human cell culture systems have gained much popularity over in vivo models, as they accurately mimic the tumor microenvironment and allow high-throughput drug screening. Of particular interest, in vitrohuman 3D tissue constructs, produced by the self-assembly method of tissue engineering, have been successfully used to model the tumor microenvironment and now represent a very promising approach to further develop diverse cancer models. In this review, we describe the importance of the tumor microenvironment and present the existing in vitro cancer models generated through the self-assembly method of tissue engineering. Lastly, we highlight the relevance of this approach to mimic various and complex tumors, including basal cell carcinoma, cutaneous neurofibroma, skin melanoma, bladder cancer, and uveal melanoma.

show abstract

“…In 2019, Carrio et al generated iPSCs from neurofibromatosis type 1 plexiform neurofibroma (PNF) cells, and those iPSCs were differentiated into neural crest cells and Schwann cells. For generating neural crest cells, they employ chemically defined medium to activate Wnt signaling while inhibiting Activin/Nodal/transforming growth factor β signaling [ 44 , 45 , 46 ], and Schwann cells were differentiated from the neural crest cells.…”

Section: Supporting Cellsmentioning

confidence: 99%

Perspectives on 3D Bioprinting of Peripheral Nerve Conduits

Soman¹,

Vijayavenkataraman

2020

IJMS

View full text Add to dashboard Cite

The peripheral nervous system controls the functions of sensation, movement and motor coordination of the body. Peripheral nerves can get damaged easily by trauma or neurodegenerative diseases. The injury can cause a devastating effect on the affected individual and his aides. Treatment modalities include anti-inflammatory medications, physiotherapy, surgery, nerve grafting and rehabilitation. 3D bioprinted peripheral nerve conduits serve as nerve grafts to fill the gaps of severed nerve bodies. The application of induced pluripotent stem cells, its derivatives and bioprinting are important techniques that come in handy while making living peripheral nerve conduits. The design of nerve conduits and bioprinting require comprehensive information on neural architecture, type of injury, neural supporting cells, scaffold materials to use, neural growth factors to add and to streamline the mechanical properties of the conduit. This paper gives a perspective on the factors to consider while bioprinting the peripheral nerve conduits.

show abstract

Reprogramming Captures the Genetic and Tumorigenic Properties of Neurofibromatosis Type 1 Plexiform Neurofibromas

Cited by 8 publications

References 0 publications

Modeling human congenital disorders with neural crest developmental defects using patient-derived induced pluripotent stem cells

Modeling human congenital disorders with neural crest developmental defects using patient-derived induced pluripotent stem cells

Human Organ-Specific 3D Cancer Models Produced by the Stromal Self-Assembly Method of Tissue Engineering for the Study of Solid Tumors

Perspectives on 3D Bioprinting of Peripheral Nerve Conduits

Contact Info

Product

Resources

About