LAUSR

Granulovacuolar degeneration (GVD) in Alzheimer’s disease (AD) involves the necrosome, which is a protein complex consisting of phosphorylated receptor‐interacting protein kinase 1 (pRIPK1), pRIPK3 and phosphorylated mixed lineage kinase domain‐like protein (pMLKL). Necrosome‐positive GVD was associated with neuron loss in AD. GVD was recently linked to the C9ORF72 mutation in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with transactive response DNA‐binding protein (TDP‐43) pathology (FTLD‐TDP). Therefore, we investigated whether GVD in cases of the ALS‐FTLD‐TDP spectrum (ALS/FTLD) shows a similar involvement of the necrosome as in AD, and whether it correlates with diagnosis, presence of protein aggregates and cell death in ALS/FTLD.