Well done to Luigi Montibeller and his supervisor Jackie de Belleroche on their research paper published in Cell Stress and Chaperones.

This is the 1st research article from the TRAINERS network with an ESR as the 1st author so congratulations to Luigi and his supervisor Jackie who are both based at Imperial College London.

The title of the article is “Amyotrophic lateral sclerosis (ALS) and Alzheimer’s disease (AD) are characterised by differential activation of ER stress pathways: focus on UPR target genes

The research identifies 44 target genes that are strongly influenced by XBP1 and ATF6.  Quantification of the expression of a subset of these genes in human post-mortem spinal cord from amyotrophic lateral sclerosis (ALS) cases and in the frontal and temporal cortex from frontotemporal lobar degeneration (FTLD) and Alzheimer’s disease (AD) cases and controls was carried out and they found that IRE1α- XBP1 and ATF6 pathways were strongly activated both in ALS and AD. In ALS,  substantial increases in target genes involved in co-chaperone activity and ER-associated degradation (ERAD) were observed while in AD  targets involved in protein folding were more prominent. The overlapping and disease-specific patterns of IRE1α-XBP1 and ATF6 target gene activation in AD and ALS  may be relevant to the development of new therapeutic strategies.