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10.1038/s41598-018-24199-0 http://scihub22266oqcxt.onion/10.1038/s41598-018-24199-0 C5899138!5899138!29654304     free     free     free Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Sci+Rep 2018 ; 8 (ä): ä Nephropedia Template TP {{tp|p=29654304|t=2018. Defective immuno- and thymoproteasome assembly causes severe immunodeficiency |pdf=|usr=}}{{29654304}} gab.com Text Defective immuno- and thymoproteasome assembly causes severe immunodeficiency JO: Sci Rep http://www.kidney.de/pget.php?&tw=1&pmid=29654304 #FOAvip By N-ethyl-N-nitrosourea (ENU) mutagenesis, we generated the mutant mouse line TUB6 that is characterised by severe combined immunodeficiency (SCID) and systemic sterile autoinflammation in homozygotes, and a selective T cell defect in heterozygotes. The causative missense point mutation results in the single amino acid exchange G170W in multicatalytic endopeptidase complex subunit-1 (MECL-1), the ?2i-subunit of the immuno- and thymoproteasome. Yeast mutagenesis and crystallographic data suggest that the severe TUB6-phenotype compared to the MECL-1 knockout mouse is caused by structural changes in the C-terminal appendage of ?2i that prevent the biogenesis of immuno- and thymoproteasomes. Proteasomes are essential for cell survival, and defective proteasome assembly causes selective death of cells expressing the mutant MECL-1, leading to the severe immunological phenotype. In contrast to the immunosubunits ?1i (LMP2) and ?5i (LMP7), mutations in the gene encoding MECL-1 have not yet been assigned to human disorders. The TUB6 mutant mouse line exemplifies the involvement of MECL-1 in immunopathogenesis and provides the first mouse model for primary immuno- and thymoproteasome-associated immunodeficiency that may also be relevant in humans. Twit Text FOAVip Defective immuno- and thymoproteasome assembly causes severe immunodeficiency ????Sci Rep http://www.kidney.de/pget.php?&tw=1&pmid=29654304 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=29654304 #FOAvip English Wikipedia <ref>{{Cite pmid|29654304}}</ref> Defective immuno- and thymoproteasome assembly causes severe immunodeficiency #MMPMID29654304 Treise I; Huber EM; Klein-Rodewald T; Heinemeyer W; Grassmann SA; Basler M; Adler T; Rathkolb B; Helming L; Andres C; Klaften M; Landbrecht C; Wieland T; Strom TM; McCoy KD; Macpherson AJ; Wolf E; Groettrup M; Ollert M; Neff F; Gailus-Durner V; Fuchs H; Hrab? de Angelis M; Groll M; Busch DH Sci Rep 2018[]; 8 (ä): ä PMID29654304show ga By N-ethyl-N-nitrosourea (ENU) mutagenesis, we generated the mutant mouse line TUB6 that is characterised by severe combined immunodeficiency (SCID) and systemic sterile autoinflammation in homozygotes, and a selective T cell defect in heterozygotes. The causative missense point mutation results in the single amino acid exchange G170W in multicatalytic endopeptidase complex subunit-1 (MECL-1), the ?2i-subunit of the immuno- and thymoproteasome. Yeast mutagenesis and crystallographic data suggest that the severe TUB6-phenotype compared to the MECL-1 knockout mouse is caused by structural changes in the C-terminal appendage of ?2i that prevent the biogenesis of immuno- and thymoproteasomes. Proteasomes are essential for cell survival, and defective proteasome assembly causes selective death of cells expressing the mutant MECL-1, leading to the severe immunological phenotype. In contrast to the immunosubunits ?1i (LMP2) and ?5i (LMP7), mutations in the gene encoding MECL-1 have not yet been assigned to human disorders. The TUB6 mutant mouse line exemplifies the involvement of MECL-1 in immunopathogenesis and provides the first mouse model for primary immuno- and thymoproteasome-associated immunodeficiency that may also be relevant in humans. äDefective immuno- and thymoproteasome assembly causes severe immunodef(epmc).pdf DeepDyve Pubget Overpricing