Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent |
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| Authors: | Chia-Yen Wu Ilsa Gómez-Curet Vicky L Funanage Mena Scavina and Wenlan Wang |
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| Institution: | (1) Department of Biological Science, University of Delaware, Newark, DE, USA;(2) Nemours Biomedical Research, Alfred I. duPont Hospital for Children, Wilmington, DE, USA;(3) Department of Pediatrics, Thomas Jefferson University, Philadelphia, PA, USA |
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| Abstract: | Background Deletion or mutation(s) of the survival motor neuron 1 (SMN1) gene causes spinal muscular atrophy (SMA). The SMN protein is known to play a role in RNA metabolism, neurite outgrowth,
and cell survival. Yet, it remains unclear how SMN deficiency causes selective motor neuron death and muscle atrophy seen
in SMA. Previously, we have shown that skin fibroblasts from SMA patients are more sensitive to the DNA topoisomerase I inhibitor
camptothecin, supporting a role for SMN in cell survival. Here, we examine the potential mechanism of camptothecin sensitivity
in SMA fibroblasts. |
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