Concurrent FOXP3- and CTLA4-associated genetic predisposition and skewed X chromosome inactivation in an autoimmune disease-prone family.

نویسندگان

  • M G Seidel
  • B Rami
  • C Item
  • E Schober
  • P Zeitlhofer
  • W D Huber
  • A Heitger
  • O A Bodamer
  • O A Haas
چکیده

CLTA4 is relevant for FOXP3(+)Treg cells, and the link between skewed X chromosome inactivation (XCI) and autoimmunity is recognized. The observation of immune dysregulation polyendocrinopathy enteropathy X-linked syndrome and multiorgan endocrine autoimmune phenomena in various members of one family, associated with a CTLA4 polymorphism and skewed XCI, provides an in vivo model of how mechanisms of immune dysregulation may cooperate.

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عنوان ژورنال:
  • European journal of endocrinology

دوره 167 1  شماره 

صفحات  -

تاریخ انتشار 2012