In vitro modelling of human proprioceptive sensory neurons in the neuromuscular system

Badiola-Mateos, Maider; Osaki, Tatsuya; Kamm, Roger D.; Samitier, J.

doi:10.1038/s41598-022-23565-3

Cited by 4 publications

(2 citation statements)

References 95 publications

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“…Results demonstrated that the cells were able to interact by the formation of annulospiral wrapping-like structures. The genetic profile comparison between healthy and ALS samples pointed to lower values of ETV1 (important for motor feedback) which highlights the involvement of proprioceptive sensory neurons in this pathology (Badiola-Mateos et al, 2022). Haberts et al also developed a brain-on-achip where microchannels connected different compartments (Figure 3D).…”

Section: Figurementioning

confidence: 97%

Advances in current in vitro models on neurodegenerative diseases

Pereira,

Lopez-Martinez,

Samitier

2023

Front. Bioeng. Biotechnol.

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Many neurodegenerative diseases are identified but their causes and cure are far from being well-known. The problem resides in the complexity of the neural tissue and its location which hinders its easy evaluation. Although necessary in the drug discovery process, in vivo animal models need to be reduced and show relevant differences with the human tissues that guide scientists to inquire about other possible options which lead to in vitro models being explored. From organoids to organ-on-a-chips, 3D models are considered the cutting-edge technology in cell culture. Cell choice is a big parameter to take into consideration when planning an in vitro model and cells capable of mimicking both healthy and diseased tissue, such as induced pluripotent stem cells (iPSC), are recognized as good candidates. Hence, we present a critical review of the latest models used to study neurodegenerative disease, how these models have evolved introducing microfluidics platforms, 3D cell cultures, and the use of induced pluripotent cells to better mimic the neural tissue environment in pathological conditions.

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Section: Figurementioning

confidence: 97%

Advances in current in vitro models on neurodegenerative diseases

Pereira,

Lopez-Martinez,

Samitier

2023

Front. Bioeng. Biotechnol.

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“…The application of spheroid models is not limited to motor neurons but extends to proprioceptive sensory neurons as well. Researchers have utilized spheroids generated from the same human neural stem cell source to compare motor neuron spheres and proprioceptive sensory spheroids in both healthy and sporadic amyotrophic lateral sclerosis (ALS) human neural stem cells 89 . By using spheroids, one can explore the distinct characteristics and disease mechanisms of different neuronal populations relevant to IPN pathogenesis (even in a specific axon region-dependent manner), providing a more comprehensive understanding of the disease.…”

Section: Common Phenotypes?mentioning

confidence: 99%

Advances and challenges in modeling inherited peripheral neuropathies using iPSCs

Van Lent,

Prior,

Pérez Siles

et al. 2024

Exp Mol Med

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Inherited peripheral neuropathies (IPNs) are a group of diseases associated with mutations in various genes with fundamental roles in the development and function of peripheral nerves. Over the past 10 years, significant advances in identifying molecular disease mechanisms underlying axonal and myelin degeneration, acquired from cellular biology studies and transgenic fly and rodent models, have facilitated the development of promising treatment strategies. However, no clinical treatment has emerged to date. This lack of treatment highlights the urgent need for more biologically and clinically relevant models recapitulating IPNs. For both neurodevelopmental and neurodegenerative diseases, patient-specific induced pluripotent stem cells (iPSCs) are a particularly powerful platform for disease modeling and preclinical studies. In this review, we provide an update on different in vitro human cellular IPN models, including traditional two-dimensional monoculture iPSC derivatives, and recent advances in more complex human iPSC-based systems using microfluidic chips, organoids, and assembloids.

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