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Disease models

Induced pluripotent stem (iPS) cell-derived astrocytes from patients with amyotrophic lateral sclerosis (ALS)

Astrocytes derived from patient iPS cells could be useful for evaluating therapeutic candidates to treat ALS. Astrocytes carrying an ALS-causing mutation in TAR DNA binding protein 43 (TDP-43; TARDBP) were generated from an iPS cell line derived from a patient with familial ALS. In culture, the astrocytes showed mislocalization of the protein in the cytoplasm and increased TDP-43 levels and decreased survival compared with astrocytes generated from control iPS cell lines. Next steps could include using the cell model to evaluate therapeutic candidates for ALS.

SciBX 6(8); doi:10.1038/scibx.2013.203
Published online Feb. 28, 2013

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Serio, A. et al. Proc. Natl. Acad. Sci. USA; published online Feb. 11, 2013;
Contact: Siddharthan Chandran, The University of Edinburgh, Edinburgh, U.K.

Contact: Tom Maniatis, Columbia University, New York, N.Y.