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10.3389/fimmu.2018.00588 http://scihub22266oqcxt.onion/10.3389/fimmu.2018.00588 C5884887!5884887 !29651288     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=29651288 &cmd=llinks): Failed to open stream: HTTP request failed! 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CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Granule Organization and Substantial Proteome Alterations in Neutrophils |pdf=|usr=}}{{29651288 }} gab.com Text CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Granule Organization and Substantial Proteome Alterations in Neutrophils JO: Front Immunol http://www.kidney.de/pget.php?&tw=1&pmid=29651288 #FOAvip 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-? (CEBPE) are one molecular etiology of the disease. Although C/EBP? 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/EBP? impacts on spatiotemporal expression and subsequent distribution of several granule proteins and further on expression of proteins controlling nuclear shape. Twit Text FOAVip CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Granule Organization and Substantial Proteome Alterations in Neutrophils ????Front Immunol http://www.kidney.de/pget.php?&tw=1&pmid=29651288 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=29651288 #FOAvip English Wikipedia <ref>{{Cite pmid|29651288 }}</ref> CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Granule Organization and Substantial Proteome Alterations in Neutrophils #MMPMID29651288 Serwas NK ; Huemer J ; Dieckmann R ; Mejstrikova E ; Garncarz W ; Litzman J ; Hoeger B ; Zapletal O ; Janda A ; Bennett KL ; Kain R ; Kerjaschky D ; Boztug K Front Immunol 2018[]; 9 (ä): 588 PMID29651288 show ga 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-? (CEBPE) are one molecular etiology of the disease. Although C/EBP? 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/EBP? impacts on spatiotemporal expression and subsequent distribution of several granule proteins and further on expression of proteins controlling nuclear shape. |*Mutation [MESH] |*Proteome [MESH] |Adult [MESH] |Biomarkers [MESH] |CCAAT-Enhancer-Binding Proteins/*genetics [MESH] |Case-Control Studies [MESH] |Child [MESH] |Child, Preschool [MESH] |Cytoplasmic Granules/immunology/metabolism [MESH] |Epitopes/immunology [MESH] |Glycoproteins/immunology/metabolism [MESH] |Humans [MESH] |Lactoferrin/*deficiency/metabolism [MESH] |Leukocyte Disorders/*etiology/*metabolism [MESH] |Membrane Glycoproteins/immunology/metabolism [MESH] |Middle Aged [MESH] |Neutrophils/immunology/*metabolism [MESH]CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Gran(epmc).pdf DeepDyve Pubget Overpricing