Junying Yuan

Keywords glucose hypometabolism necroptosis p-Tau neurodegeneration Get full text access Log in, subscribe or purchase for full access. References 1. Arriagada, P.V. ∙ Growdon, J.H. ∙ Hedley-Whyte, E.T. ... Neurofibrillary tangles but not senile plaques parallel duration and severity of Alzheimer's disease Neurology. 1992; 42:631-639 2. Riley, K.P. ∙ Snowdon, D.A. ∙ Markesbery, W.R. Alzheimer's neurofibrillary pathology and the spectrum of cognitive function: findings from the Nun Study Ann. Neurol.

Abstract Dynamic assembly of the complex I signalosome mediated by three death domain (DD)-containing proteins—TNFR1, TRADD and RIPK1—is key for transmitting extracellular TNF stimuli to intracellular NF-κB signalling in controlling ‘live or die’ cell fate1.

Abstract Systemic autoinflammatory diseases (SAID) with inborn errors of cell death (IECD) are caused by overactivation of programmed cell death (PCD). However, the pathogenesis by which PCD leads to autoinflammation remains unclear. Here, we identified IECD patients carrying compound heterozygous RIPK1 variants K377E/R390G with autoinflammatory manifestations. Mechanistically, K377E and R390G mutations suppress NF-κB signaling and activate RIPK1 to promote cell death.