Nature - USA (2020-01-02)
Nature | Vol 577 | 2 January 2020 | 109 Article A dominant autoinflammatory disease caused by non-cleavable variants of RIPK1 Pa ...
110 | Nature | Vol 577 | 2 January 2020 Article Patients with RIPK1 non-cleavable variants The first patient (P1) is a two-year- ...
Nature | Vol 577 | 2 January 2020 | 111 cells (Fig. 3b). Notably, patient PBMCs treated with TNF and SM-164 showed increased lev ...
112 | Nature | Vol 577 | 2 January 2020 Article We also examined the effect of non-cleavable RIPK1 on ligands of other death rec ...
Nature | Vol 577 | 2 January 2020 | 113 Necroptosis and ferroptosis resistance in fibroblasts Notably, we observed the opposite ...
114 | Nature | Vol 577 | 2 January 2020 Article Discussion Our study identified a dominantly inherited autoinflammatory disease ...
Methods Patients Patient P1 was evaluated under protocols approved by the Insti- tutional Review Board by Children’s Hospital of ...
Article Fisher, 78442) and 10% glycerol) for 10 min and centrifuged at 20,000g for 10 min. Protein concentration was measured on ...
Science Foundation (31530041). The work of H.L. was supported by Ningbo Health Branding Subject Fund (PPXK2018-06). Author contr ...
Article Extended Data Fig. 1 | See next page for caption. ...
Extended Data Fig. 1 | Identif ication of RIPK1 variants and their effects. a, Schematic of the WES data-filtering approach unde ...
Article Extended Data Fig. 2 | See next page for caption. ...
Extended Data Fig. 2 | Increased pro-inf lammatory signalling in patient PBMCs. a, Intracellular cytokine staining of patient PB ...
Article Extended Data Fig. 3 | Increased expression of genes in NF-κB and type-I IFN pathways in patient PBMCs. a, Visualization ...
Extended Data Fig. 4 | Patient PBMCs are sensitive to TNF-induced cell death but not to ferroptosis. a, qPCR of PBMCs confirmed ...
Article Extended Data Fig. 5 | See next page for caption. ...
Extended Data Fig. 5 | RIPK1 cleavage site variants in MEFs promote cell death and inf lammatory response but have no protective ...
Article Extended Data Fig. 6 | Patient f ibroblasts were resistant to both necroptosis and ferroptosis. a, Patient fibroblasts w ...
Extended Data Table 1 | Clinical manifestations of patients with RIPK1 variants ...
Article Extended Data Table 2 | The count and percentage of T and B cells in patient 1 The relative and absolute numbers of lymp ...
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