Article
Short telomere syndromes cause a primary T cell immunodeficiency.
The Journal of clinical investigation - 3 Dec 2018
Wagner Christa L, Hanumanthu Vidya Sagar, Talbot C Conover, Abraham Roshini S, Hamm David, Gable Dustin L, Kanakry Christopher G, Applegate Carolyn D, Siliciano Janet, Jackson J Brooks, Desiderio Stephen, Alder Jonathan K, Luznik Leo, Armanios Mary
Abstract excerpt
The mechanisms that drive T cell aging are not understood. We report that children and adult telomerase mutation carriers with short telomere length (TL) develop a T cell immunodeficiency that can manifest in the absence of bone marrow failure and causes life-threatening opportunistic infections. Mutation carriers shared T cell-aging phenotypes seen in adults 5 decades older, including depleted naive T cells,...
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