Interestingly, we had previously identified in pure motoneurones of two mouse models of motoneurone disease (hSOD1G93A and pmn) an increase in IGFBP. Also, an IGFBP that binds to IGF-1 and IGF-2 (nephroblastoma over-expressed gene) was up-regulated at all disease stages in hSOD1G93A mice [8] and IGFBP4 mRNA was induced at pre-symptomatic age in pmn mice [9]. Here, IGFBP4 is linked to Nephroblastoma.