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- Neehus, AL, et al.
(författare)
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Impaired respiratory burst contributes to infections in PKCδ-deficient patients
- 2021
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Ingår i: The Journal of experimental medicine. - : Rockefeller University Press. - 1540-9538 .- 0022-1007. ; 218:9
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Tidskriftsartikel (refereegranskat)abstract
- Patients with autosomal recessive protein kinase C δ (PKCδ) deficiency suffer from childhood-onset autoimmunity, including systemic lupus erythematosus. They also suffer from recurrent infections that overlap with those seen in patients with chronic granulomatous disease (CGD), a disease caused by defects of the phagocyte NADPH oxidase and a lack of reactive oxygen species (ROS) production. We studied an international cohort of 17 PKCδ-deficient patients and found that their EBV-B cells and monocyte-derived phagocytes produced only small amounts of ROS and did not phosphorylate p40phox normally after PMA or opsonized Staphylococcus aureus stimulation. Moreover, the patients’ circulating phagocytes displayed abnormally low levels of ROS production and markedly reduced neutrophil extracellular trap formation, altogether suggesting a role for PKCδ in activation of the NADPH oxidase complex. Our findings thus show that patients with PKCδ deficiency have impaired NADPH oxidase activity in various myeloid subsets, which may contribute to their CGD-like infectious phenotype.
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- Serwas, N. K., et al.
(författare)
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CEBPE-mutant specific granule deficiency correlates with aberrant granule organization and substantial proteome alterations in neutrophils
- 2018
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Ingår i: Frontiers in Immunology. - : Frontiers Media SA. - 1664-3224. ; 9:MAR
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Tidskriftsartikel (refereegranskat)abstract
- Specific granule deficiency (SGD) is a rare disorder characterized by abnormal neutrophils evidenced by reduced granules, absence of granule proteins, and atypical bilobed nuclei. Mutations in CCAAT/enhancer-binding protein-e (CEBPE) are one molecular etiology of the disease. Although C/EBPe has been studied extensively, the impact of CEBPE mutations on neutrophil biology remains elusive. Here, we identified two SGD patients bearing a previously described heterozygous mutation (p.Val218Ala) in CEBPE. We took this rare opportunity to characterize SGD neutrophils in terms of granule distribution and protein content. Granules of patient neutrophils were clustered and polarized, suggesting that not only absence of specific granules but also defects affecting other granules contribute to the phenotype. Our analysis showed that remaining granules displayed mixed protein content and lacked several glycoepitopes. To further elucidate the impact of mutant CEBPE, we performed detailed proteomic analysis of SGD neutrophils. Beside an absence of several granule proteins in patient cells, we observed increased expression of members of the linker of nucleoskeleton and cytoskeleton complex (nesprin-2, vimentin, and lamin-B2), which control nuclear shape. This suggests that absence of these proteins in healthy individuals might be responsible for segmented shapes of neutrophilic nuclei. We further show that the heterozygous mutation p.Val218Ala in CEBPE causes SGD through prevention of nuclear localization of the protein product. In conclusion, we uncover that absence of nuclear C/EBPe impacts on spatiotemporal expression and subsequent distribution of several granule proteins and further on expression of proteins controlling nuclear shape. © 2018 Serwas, Huemer, Dieckmann, Mejstrikova, Garncarz, Litzman, Hoeger, Zapletal, Janda, Bennett, Kain, Kerjaschky and Boztug.
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- Grunert, K.G., et al.
(författare)
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Perception of health claims among nordic consumers
- 2009
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Ingår i: Journal of Consumer Policy. - : Springer Science and Business Media LLC. - 0168-7034 .- 1573-0700. ; 32:3, s. 269-287
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Tidskriftsartikel (refereegranskat)abstract
- Health claim perception was investigated by a web-based instrument with a sample of 4,612 respondents in the Nordic countries (Denmark, Finland, Iceland, Norway, Sweden). Respondents decided which of a pair of claims sounded better, was easier to understand and was more convincing in their opinion. Claims were constructed from an underlying universe combining different active ingredients (familiar, unfamiliar), type of claim (combination of information about ingredient, physiological function and health benefit), framing (positive, negative), and use of qualifier (with, without "may"). Across pairs, the claims differed in the health benefit addressed (cardiovascular health, dementia, bodyweight). Results showed that respondents could be grouped into two roughly equally sized classes that differed in the type of claim preferred: one class prefers "long" claims that give the full story consisting of active ingredient, physiological function and health benefit, whereas the other prefers "short" claims consisting of the health benefit only. Results also showed that the familiar ingredient is preferred to the unfamiliar one, whereas effects of positive versus negative framing depended on the type of health benefit addressed. © Springer Science+Business Media, LLC. 2009.
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