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10.1073/pnas.1709111115

http://scihub22266oqcxt.onion/10.1073/pnas.1709111115
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suck abstract from ncbi


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pmid29305555      Proc+Natl+Acad+Sci+U+S+A 2018 ; 115 (4): E705-14
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  • Impaired lymphoid extracellular matrix impedes antibacterial immunity in epidermolysis bullosa #MMPMID29305555
  • Nyström A; Bornert O; Kühl T; Gretzmeier C; Thriene K; Dengjel J; Pfister-Wartha A; Kiritsi D; Bruckner-Tuderman L
  • Proc Natl Acad Sci U S A 2018[Jan]; 115 (4): E705-14 PMID29305555show ga
  • We describe a unique role for the lymphoid extracellular matrix in maintaining systemic innate immunity. Our findings are based on studies of the genetic skin disorder recessive dystrophic epidermolysis bullosa in which affected individuals display dramatically increased bacterial colonization of skin and mucosa. We show that the increased colonization is a consequence of loss of the protein at fault?collagen VII?from the lymphoid extracellular matrix. Our study describes an intrinsic innate immune defect resulting from a genetic connective tissue disease. Hence, the data will have broad implications for deciphering the hitherto-underestimated regulatory role of the extracellular matrix in lymphoid organs and for the understanding of pathomechanisms in disorders of the extracellular matrix.
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