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pro vyhledávání: '"Vivien R. Sutton"'
Severely impaired CTL killing is a feature of the neurological disorder Niemann-Pick disease type C1
Autor:
Daniela Castiblanco, Jesse A. Rudd-Schmidt, Tahereh Noori, Vivien R. Sutton, Ya Hui Hung, Thijs W. H. Flinsenberg, Adrian W. Hodel, Neil D. Young, Nicholas Smith, Drago Bratkovic, Heidi Peters, Mark Walterfang, Joseph A. Trapani, Amelia J. Brennan, Ilia Voskoboinik
Publikováno v:
Blood. 139:1833-1849
Niemann-Pick disease type C1 (NP-C1) is a rare lysosomal storage disorder resulting from mutations in an endolysosomal cholesterol transporter, NPC1. Despite typically presenting with pronounced neurological manifestations, NP-C1 also resembles long-
Autor:
Angus P. R. Johnston, Ruby H. P. Law, Joseph A. Trapani, James C. Whisstock, Helen R. Saibil, Jamie A. Lopez, Catherina H. Bird, Natalya Lukoyanova, Olivia Susanto, Misty R. Jenkins, Vivien R. Sutton, Ilia Voskoboinik, Phillip I. Bird
Publikováno v:
Blood. 121:2659-2668
Cytotoxic lymphocytes serve a key role in immune homeostasis by eliminating virus-infected and transformed target cells through the perforin-dependent delivery of proapoptotic granzymes. However, the mechanism of granzyme entry into cells remains unr
Autor:
Colin M. House, Joseph A. Trapani, Jenny Chia, Annette Ciccone, Ilia Voskoboinik, Phillip K. Darcy, Hideo Yagita, Vivien R. Sutton
Publikováno v:
Blood. 110:1184-1190
Perforin (PRF), a pore-forming protein expressed in cytotoxic lymphocytes, plays a key role in immune surveillance and immune homeostasis. The A91V substitution has a prevalence of 8% to 9% in population studies. While this variant has been suspected