Establishment of iPS cells from amyotrophic lateral sclerosis model mice and motor neuronal differentiation

Abstract

ubject:Tounravelpathogenesis of amyotrophic lateral sclerosis (ALS),methds using induced pluripotent stem cells (iPS cells) are promising. We stablish iPS cells from ALS model mice, mutant superoxide dismutase1 SOD1) transgenic mice, induce neural differentiation and unravel pathoenesis of ALS. ethods: We introduced known four factors (Oct3/4, Sox2, Klf4, c-Myc) into ouse embryonic fibroblasts obtained from crossbreeding of SOD1G93A mice ith Nanog-GFP-IRES-Puror mice using retroviral vectors and cultured them n SNL feeder cells. We picked ES cell-like colonies, cultured them and conrmed establishment of iPS cells using ES cell markers. Then we induced irected differentiation using retinoic acid and Smoothened agonist and conrmed motor neuronal identity by immunocytochemistry. esults: We established iPS cells from mutant SOD1 mice and control mice nd confirmed motor neuronal differentiation. onclusions: These results indicate that iPS cells fromALSmodelmice possess roperties of ES cellswithmutant SOD1andmay contribute to establishment f ALS model(s) in vitro. esearch fund: Health and Labour Sciences Research Grants.