Figure 1. Chest CT scan and laboratory data from the patient. (A) Chest CT scanners of the patient at the start of her respiratory symptoms (left), referral to our center (middle), and 38 days after immunoglobulin replacement therapy initiation More about this image found in Chest CT scan and laboratory data from the patient. (A) Chest CT scanners ...

Figure 1. CTLA-4 Y218* mutation in four generations of a family. (A) Pedigree of the affected family, with proband identified by the arrow (patient 16); filled symbols signify clinically affected family members, with the heterozygous CTLA4 More about this image found in CTLA-4 Y218* mutation in four generations of a family. (A) Pedigree of the...

Figure 2. CTLA-4 Y218* mutation leads to reduced protein levels in primary patient cells. (A) Representative histograms of CTLA-4 mean fluorescence intensity (MFI) levels in T_reg cells in one healthy donor and P16 with p.(Tyr218*) variant after More about this image found in CTLA-4 Y218* mutation leads to reduced protein levels in primary patient ce...

Figure 3. CTLA-4 Y218* mutation leads to protein instability. (A–C) (A) Representative histogram showing MFI of CTLA-4 levels in Jurkat cells with WT or Y218* CTLA-4 treated with CHX to block protein translation, Baf to block lysosome More about this image found in CTLA-4 Y218* mutation leads to protein instability. (A–C) (A) Representati...

Figure 4. CTLA-4 Y218* mutation leads to dysfunctional TE. (A–C) (A) Experimental design and (B) representative dot plot of CD80 donor levels following TE by Jurkat recipient cells with WT (blue blots) or Y218* CTLA-4 mutation (red blots), with More about this image found in CTLA-4 Y218* mutation leads to dysfunctional TE. (A–C) (A) Experimental de...

Figure 1. Clinical and immunophenotyping data for hospitalized patients compared to controls. (A) Clinical data from the “all hospital,” severe, and mild cohort. Data expressed as frequencies, mean (standard deviation), or mode (interquartile More about this image found in Clinical and immunophenotyping data for hospitalized patients compared to c...

Figure 1. Clinical and genetic findings in a 4-year-old patient with RSV-induced fever and seizures. (A) Chest x-ray at day 1 of hospital admission showing pulmonary infiltration with fluid and atelectasis of the right upper lobe. (B) Magnetic More about this image found in Clinical and genetic findings in a 4-year-old patient with RSV-induced feve...

Figure 2. Hyperinflammatory cytokine and IFN responses in patient PBMCs. (A–H) PBMCs from the patient (red) and three controls (grey) were left untreated (UT), stimulated with TLR4 agonist LPS, TLR7/8 agonist R848, transfected with PolyIC for More about this image found in Hyperinflammatory cytokine and IFN responses in patient PBMCs. (A–H) PBMCs...

Figure S1. Inflammatory cytokine and IFN responses in PBMCs following RSV infection. PBMCs from the patient (P, red) and three controls (C, grey) were mock infected (mock) or infected with RSV (RSV-A strain Long, MOI 1). (A–G) Cells were More about this image found in Inflammatory cytokine and IFN responses in PBMCs following RSV infection. ...

Figure 3. Impaired RSV-induced apoptotic cell death and reduced autophagy in patient primary fibroblasts. (A and B) Whole cell lysates from dermal fibroblasts from three controls (C, grey) and the patient (P, red) were analyzed for More about this image found in Impaired RSV-induced apoptotic cell death and reduced autophagy in patient ...

Figure S2. Quantification of apoptotic and necrotic cells in patient and control fibroblasts. (A–D) Representative density plots showing the analysis of noninflammatory apoptotic and inflammatory necrotic cells by flow cytometry. (A and B) More about this image found in Quantification of apoptotic and necrotic cells in patient and control fibro...

Figure S3. Generation of PARK7-deficient A549 and SH-SY5Y cells and cytokine responses to RSV infection. (A and B) Immunoblot and RT-PCR demonstrating absence of RSV replication in primary fibroblasts. Primary fibroblasts from a healthy donor More about this image found in Generation of PARK7-deficient A549 and SH-SY5Y cells and cytokine responses...

Figure 4. Enhanced cellular stress pathways and hyperinflammatory cytokine responses in PARK7-deficient neuronal SH-SY5Y cells. (A) Human pulmonary A594 cells deficient in PARK7 (red) and control AAVS1^KO (grey) were mock treated or infected with More about this image found in Enhanced cellular stress pathways and hyperinflammatory cytokine responses ...

Figure 5. Illustration of the working hypothesis on dysregulated responses to viral infection and damage by oxidative stress in PARK7 deficiency. In healthy individuals with PARK7-sufficient cells (left), viral infection activates PPRs More about this image found in Illustration of the working hypothesis on dysregulated responses to viral i...