Dataset Information


Gene expression profiling of LMC and MMC motor neurons in SMA mice

ABSTRACT: Spinal muscular atrophy (SMA) is a neurodegenerative disease which exhibits selective motor neuron death caused by a ubiquitous deficiency of the survival motor neuron (SMN) protein. It remains unclear how the ubiquitous reduction of SMN lead to death in selective motor neuron pools. Medial motor neuron columns (MMC) are vulnerable, whereas lateral motor columns (LMC) are resistant to motor neuron death in SMA. Here we performed microarray and pathway analysis comparing cholera toxin subunit B (CTb) labeled vulnerable MMC and resistant LMC of pre-symptomatic SMA with corresponding motor neuron columns of control mice to identify pathways involved in selective motor neuron death in SMA. WT is FVB. SMN is Delta7 (SMNΔ7;SMN2;Smn-) on a FVB background. Overall design: 8 different groups (WT and SMA medial motor columns (MMC),WT and SMA lateral motor columns (LMC), WT and SMA DRG (Dorsal Root Ganglion) and WT and SMA Raphe. 3 biological replicates per group.

INSTRUMENT(S): Illumina MouseWG-6 v2.0 expression beadchip

SUBMITTER: George Z Mentis 

PROVIDER: GSE81245 | GEO | 2017-12-12



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The hallmark of spinal muscular atrophy (SMA), an inherited disease caused by ubiquitous deficiency in the SMN protein, is the selective degeneration of subsets of spinal motor neurons. Here, we show that cell-autonomous activation of p53 occurs in vulnerable but not resistant motor neurons of SMA mice at pre-symptomatic stages. Moreover, pharmacological or genetic inhibition of p53 prevents motor neuron death, demonstrating that induction of p53 signaling drives neurodegeneration. At late disea  ...[more]

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