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Article

An Example of Neuro-Glial Commitment and Differentiation of Muse Stem Cells Obtained from Patients with IQSEC2-Related Neural Disorder: A Possible New Cell-Based Disease Model

by
Sura Hilal Ahmed Al Sammarraie
1,
Domenico Aprile
1,
Ilaria Meloni
2,
Nicola Alessio
1,
Francesca Mari
2,
Marianna Manata
2,
Caterina Lo Rizzo
2,
Giovanni Di Bernardo
1,3,
Gianfranco Peluso
4,
Alessandra Renieri
2,* and
Umberto Galderisi
1,3,5,*
1
Department of Experimental Medicine, Luigi Vanvitelli Campania University, 80138 Naples, Italy
2
Department of Medical Biotechnology, University of Siena, 53100 Siena, Italy
3
Sbarro Institute for Cancer Research and Molecular Medicine, Center for Biotechnology, Temple University, Philadelphia, PA 19122, USA
4
UniCamillus, 00131 Rome, Italy
5
Genome and Stem Cell Center (GENKÖK), Erciyes University, 38280 Kayseri, Turkey
*
Authors to whom correspondence should be addressed.
Cells 2023, 12(7), 977; https://doi.org/10.3390/cells12070977
Submission received: 3 March 2023 / Revised: 13 March 2023 / Accepted: 21 March 2023 / Published: 23 March 2023
(This article belongs to the Special Issue Importance of Primary Cell Culture in Biomedical Research)
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Abstract

Although adult stem cells may be useful for studying tissue-specific diseases, they cannot be used as a general model for investigating human illnesses given their limited differentiation potential. Multilineage-differentiating stress-enduring (Muse) stem cells, a SSEA3(+) cell population isolated from mesenchymal stromal cells, fat, and skin fibroblasts, may be able to overcome that restriction. The Muse cells present in fibroblast cultures obtained from biopsies of patients’ skin may be differentiated into cells of interest for analyzing diseases. We isolated Muse stem cells from patients with an intellectual disability (ID) and mutations in the IQSEC2 gene (i.e., BRAG1 gene) and induced in vitro neuroglial differentiation to study cell commitment and the differentiation of neural lineages. The neuroglial differentiation of Muse cells revealed that IQSEC2 mutations may alter the self-renewal and lineage specification of stem cells. We observed a decrease in the percentage of SOX2 (+) neural stem cells and neural progenitors (i.e., SOX2+ and NESTIN+) in cultures obtained from Muse cells with the mutated IQSEC2 gene. The alteration in the number of stem cells and progenitors produced a bias toward the astrocytes’ differentiation. Our research demonstrates that Muse stem cells may represent a new cell-based disease model.
Keywords: intellectual disability; disease model; adult stem cells intellectual disability; disease model; adult stem cells

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MDPI and ACS Style

Al Sammarraie, S.H.A.; Aprile, D.; Meloni, I.; Alessio, N.; Mari, F.; Manata, M.; Lo Rizzo, C.; Di Bernardo, G.; Peluso, G.; Renieri, A.; et al. An Example of Neuro-Glial Commitment and Differentiation of Muse Stem Cells Obtained from Patients with IQSEC2-Related Neural Disorder: A Possible New Cell-Based Disease Model. Cells 2023, 12, 977. https://doi.org/10.3390/cells12070977

AMA Style

Al Sammarraie SHA, Aprile D, Meloni I, Alessio N, Mari F, Manata M, Lo Rizzo C, Di Bernardo G, Peluso G, Renieri A, et al. An Example of Neuro-Glial Commitment and Differentiation of Muse Stem Cells Obtained from Patients with IQSEC2-Related Neural Disorder: A Possible New Cell-Based Disease Model. Cells. 2023; 12(7):977. https://doi.org/10.3390/cells12070977

Chicago/Turabian Style

Al Sammarraie, Sura Hilal Ahmed, Domenico Aprile, Ilaria Meloni, Nicola Alessio, Francesca Mari, Marianna Manata, Caterina Lo Rizzo, Giovanni Di Bernardo, Gianfranco Peluso, Alessandra Renieri, and et al. 2023. "An Example of Neuro-Glial Commitment and Differentiation of Muse Stem Cells Obtained from Patients with IQSEC2-Related Neural Disorder: A Possible New Cell-Based Disease Model" Cells 12, no. 7: 977. https://doi.org/10.3390/cells12070977

APA Style

Al Sammarraie, S. H. A., Aprile, D., Meloni, I., Alessio, N., Mari, F., Manata, M., Lo Rizzo, C., Di Bernardo, G., Peluso, G., Renieri, A., & Galderisi, U. (2023). An Example of Neuro-Glial Commitment and Differentiation of Muse Stem Cells Obtained from Patients with IQSEC2-Related Neural Disorder: A Possible New Cell-Based Disease Model. Cells, 12(7), 977. https://doi.org/10.3390/cells12070977

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