Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons. / Boza-Morán, Maria G; Martínez-Hernández, Rebeca; Bernal, Sara; Wanisch, Klaus; Also-Rallo, Eva; le Heron, Anita; Alías, Laura; Denis, Cécile; Girard, Mathilde; Yee, Jiing-Kuan; Tizzano, Eduardo F; Yáñez-Muñoz, Rafael J.

In: Scientific Reports, Vol. 5, 11696, 26.06.2015, p. 1-17.

Research output: Contribution to journalArticlepeer-review

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Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons. / Boza-Morán, Maria G; Martínez-Hernández, Rebeca; Bernal, Sara; Wanisch, Klaus; Also-Rallo, Eva; le Heron, Anita; Alías, Laura; Denis, Cécile; Girard, Mathilde; Yee, Jiing-Kuan; Tizzano, Eduardo F; Yáñez-Muñoz, Rafael J.

In: Scientific Reports, Vol. 5, 11696, 26.06.2015, p. 1-17.

Research output: Contribution to journalArticlepeer-review

Harvard

Boza-Morán, MG, Martínez-Hernández, R, Bernal, S, Wanisch, K, Also-Rallo, E, le Heron, A, Alías, L, Denis, C, Girard, M, Yee, J-K, Tizzano, EF & Yáñez-Muñoz, RJ 2015, 'Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons', Scientific Reports, vol. 5, 11696, pp. 1-17. https://doi.org/10.1038/srep11696

APA

Boza-Morán, M. G., Martínez-Hernández, R., Bernal, S., Wanisch, K., Also-Rallo, E., le Heron, A., Alías, L., Denis, C., Girard, M., Yee, J-K., Tizzano, E. F., & Yáñez-Muñoz, R. J. (2015). Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons. Scientific Reports, 5, 1-17. [11696]. https://doi.org/10.1038/srep11696

Vancouver

Boza-Morán MG, Martínez-Hernández R, Bernal S, Wanisch K, Also-Rallo E, le Heron A et al. Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons. Scientific Reports. 2015 Jun 26;5:1-17. 11696. https://doi.org/10.1038/srep11696

Author

Boza-Morán, Maria G ; Martínez-Hernández, Rebeca ; Bernal, Sara ; Wanisch, Klaus ; Also-Rallo, Eva ; le Heron, Anita ; Alías, Laura ; Denis, Cécile ; Girard, Mathilde ; Yee, Jiing-Kuan ; Tizzano, Eduardo F ; Yáñez-Muñoz, Rafael J. / Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons. In: Scientific Reports. 2015 ; Vol. 5. pp. 1-17.

BibTeX

@article{d45a86b25edb49f7adc9cfa611400632,
title = "Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons",
abstract = "Spinal muscular atrophy (SMA) is a neuromuscular disease caused by mutations in Survival Motor Neuron 1 (SMN1), leading to degeneration of alpha motor neurons (MNs) but also affecting other cells. Induced pluripotent stem cell (iPSC)-derived human MN models from severe SMA patients have shown relevant phenotypes. We have produced and fully characterized iPSCs from members of a discordant consanguineous family with chronic SMA. We differentiated the iPSC clones into ISL-1+/ChAT+ MNs and performed a comparative study during the differentiation process, observing significant differences in neurite length and number between family members. Analyses of samples from wild-type, severe SMA type I and the type IIIa/IV family showed a progressive decay in SMN protein levels during iPSC-MN differentiation, recapitulating previous observations in developmental studies. PLS3 underwent parallel reductions at both the transcriptional and translational levels. The underlying, progressive developmental decay in SMN and PLS3 levels may lead to the increased vulnerability of MNs in SMA disease. Measurements of SMN and PLS3 transcript and protein levels in iPSC-derived MNs show limited value as SMA biomarkers.",
author = "Boza-Mor{\'a}n, {Maria G} and Rebeca Mart{\'i}nez-Hern{\'a}ndez and Sara Bernal and Klaus Wanisch and Eva Also-Rallo and {le Heron}, Anita and Laura Al{\'i}as and C{\'e}cile Denis and Mathilde Girard and Jiing-Kuan Yee and Tizzano, {Eduardo F} and Y{\'a}{\~n}ez-Mu{\~n}oz, {Rafael J}",
year = "2015",
month = jun,
day = "26",
doi = "10.1038/srep11696",
language = "English",
volume = "5",
pages = "1--17",
journal = "Scientific Reports",
issn = "2045-2322",
publisher = "Nature Publishing Group",

}

RIS

TY - JOUR

T1 - Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons

AU - Boza-Morán, Maria G

AU - Martínez-Hernández, Rebeca

AU - Bernal, Sara

AU - Wanisch, Klaus

AU - Also-Rallo, Eva

AU - le Heron, Anita

AU - Alías, Laura

AU - Denis, Cécile

AU - Girard, Mathilde

AU - Yee, Jiing-Kuan

AU - Tizzano, Eduardo F

AU - Yáñez-Muñoz, Rafael J

PY - 2015/6/26

Y1 - 2015/6/26

N2 - Spinal muscular atrophy (SMA) is a neuromuscular disease caused by mutations in Survival Motor Neuron 1 (SMN1), leading to degeneration of alpha motor neurons (MNs) but also affecting other cells. Induced pluripotent stem cell (iPSC)-derived human MN models from severe SMA patients have shown relevant phenotypes. We have produced and fully characterized iPSCs from members of a discordant consanguineous family with chronic SMA. We differentiated the iPSC clones into ISL-1+/ChAT+ MNs and performed a comparative study during the differentiation process, observing significant differences in neurite length and number between family members. Analyses of samples from wild-type, severe SMA type I and the type IIIa/IV family showed a progressive decay in SMN protein levels during iPSC-MN differentiation, recapitulating previous observations in developmental studies. PLS3 underwent parallel reductions at both the transcriptional and translational levels. The underlying, progressive developmental decay in SMN and PLS3 levels may lead to the increased vulnerability of MNs in SMA disease. Measurements of SMN and PLS3 transcript and protein levels in iPSC-derived MNs show limited value as SMA biomarkers.

AB - Spinal muscular atrophy (SMA) is a neuromuscular disease caused by mutations in Survival Motor Neuron 1 (SMN1), leading to degeneration of alpha motor neurons (MNs) but also affecting other cells. Induced pluripotent stem cell (iPSC)-derived human MN models from severe SMA patients have shown relevant phenotypes. We have produced and fully characterized iPSCs from members of a discordant consanguineous family with chronic SMA. We differentiated the iPSC clones into ISL-1+/ChAT+ MNs and performed a comparative study during the differentiation process, observing significant differences in neurite length and number between family members. Analyses of samples from wild-type, severe SMA type I and the type IIIa/IV family showed a progressive decay in SMN protein levels during iPSC-MN differentiation, recapitulating previous observations in developmental studies. PLS3 underwent parallel reductions at both the transcriptional and translational levels. The underlying, progressive developmental decay in SMN and PLS3 levels may lead to the increased vulnerability of MNs in SMA disease. Measurements of SMN and PLS3 transcript and protein levels in iPSC-derived MNs show limited value as SMA biomarkers.

U2 - 10.1038/srep11696

DO - 10.1038/srep11696

M3 - Article

VL - 5

SP - 1

EP - 17

JO - Scientific Reports

JF - Scientific Reports

SN - 2045-2322

M1 - 11696

ER -