Transcription profiling of spinal cords from nmd mice tranplanted with ALDHhiSSClo neural stem cells to study molecular mechanism of delayed SMARD1 disease progression induced by the transplantation.

ABSTRACT: SMARD1 is an infantile autosomal recessive motor neuron (MN) disease, caused by mutations in the Immunoglobulin mu binding protein 2 (IGHMBP2). We investigated the potential of a spinal cord neural stem cell population isolated on the basis of aldehyde dehydrogenase activity (ALDH) to modify disease progression of nmd mice, an animal model of SMARD1. ALDHhiSSClo stem cells are self-renewing and multipotent and when intratechally transplanted in nmd mice generate motor neurons properly localized in the spinal cord ventral horns. Transplanted nmd animals presented delayed disease progression, sparing of motor neurons and ventral root axons and increased life-span. To further investigate the molecular events responsible for these differences, microarray and Real time RT-PCR analysis of wild-

ORGANISM(S): Mus musculus

DISEASE(S): SMARD1

SUBMITTER: Stefania Corti 

PROVIDER: E-GEOD-3075 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress