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

被引：3

作者：

Al Sammarraie, Sura Hilal Ahmed ^{[1
]}

Aprile, Domenico ^{[1
]}

Meloni, Ilaria ^{[2
]}

Alessio, Nicola ^{[1
]}

Mari, Francesca ^{[2
]}

Manata, Marianna ^{[2
]}

Lo Rizzo, Caterina ^{[2
]}

Di Bernardo, Giovanni ^{[1
,3
]}

Peluso, Gianfranco ^{[4
]}

Renieri, Alessandra ^{[2
]}

Galderisi, Umberto ^{[1
,3
,5
]}

机构：

[1] Luigi Vanvitelli Campania Univ, Dept Expt Med, I-80138 Naples, Italy

[2] Univ Siena, Dept Med Biotechnol, I-53100 Siena, Italy

[3] Temple Univ, Sbarro Inst Canc Res & Mol Med, Ctr Biotechnol, Philadelphia, PA 19122 USA

[4] UniCamillus, I-00131 Rome, Italy

[5] Erciyes Univ, Genome & Stem Cell Ctr GENKOK, TR-38280 Kayseri, Turkiye

来源：

CELLS | 2023年 / 12卷 / 07期

关键词：

intellectual disability; disease model; adult stem cells; EPIGENETIC MEMORY; GENERATION; OCT4; SOX2;

D O I：

10.3390/cells12070977

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

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.

引用

页数：14