HOW THE IMMUNE SYSTEM CONTROLS VIRAL INFECTIONS AND CANCER

Severely impaired CTL killing is a feature of the neurological disorder Niemann-Pick disease type C1

Daniela Castiblanco, Jesse A Rudd-Schmidt, Tahereh Noori, Vivien R Sutton, Ya Hui Hung, Thijs WH Flinsenberg, Adrian W Hodel, Neil D Young, Nicholas Smith, Drago Bratkovic, Heidi Peters, Mark Walterfang, Joseph A Trapani, Amelia J Brennan, Ilia Voskoboinik

2022-03-24

Niemann-Pick disease type C1 (NP-C1) is a rare lysosomal storage disorder resulting from mutations in an endolysosomal cholesterol..

Lipid specificity of the immune effector perforin

Adrian W Hodel, Jesse A Rudd-Schmidt, Joseph A Trapani, Ilia Voskoboinik, Bart W Hoogenboom

2021-12-24

Perforin is a pore forming protein used by cytotoxic T lymphocytes to remove cancerous or virus-infected cells during the immune r..

Recovery of natural killer cell cytotoxicity in a p.A91V perforin homozygous patient following severe haemophagocytic lymphohistiocytosis

Helena S Jang, Thijs WH Flinsenberg, Paul Lacaze, Kevin YT Thia, Tahereh Noori, Suran L Fernando, Ian Kerridge, Moeen Riaz, John J McNeil, Piers A Blombery, Joseph A Trapani, Ilia Voskoboinik

2020-04-27

Prevalence and disease predisposition of p.A91V perforin in an aged population of European ancestry

Ilia Voskoboinik, Paul Lacaze, Helena Sung-In Jang, Thijs Flinsenberg, Suran L Fernando, Ian Kerridge, Moeen Riaz, Robert Sebra, Kevin Thia, Taherah Noori, Eric E Schadt, John J McNeil, Joseph A Trapani

2020-02-20

<jats:p>In a population-based analysis including a large database restricted to patients over age 70, the authors demonstrate that..

Lipid order and charge protect killer T cells from accidental death

Jesse A Rudd-Schmidt, Adrian W Hodel, Tahereh Noori, Jamie A Lopez, Hyun-Jung Cho, Sandra Verschoor, Annette Ciccone, Joseph A Trapani, Bart W Hoogenboom, Ilia Voskoboinik

2019-11-27

Killer T cells (cytotoxic T lymphocytes, CTLs) maintain immune homoeostasis by eliminating virus-infected and cancerous cells. CTL..

Neonatal Cytomegalovirus Palatal Ulceration and Bocavirus Pneumonitis Associated With a Defect of Lymphocyte Cytotoxicity Caused by Mutations in UNC13D

Paul Edgar Gray, Bella Shadur, Susan Russell, Richard Mitchell, Kerri Gallagher, Kevin Thia, Pamela Palasanthiran, Ilia Voskoboinik

2019-03-01

Single gene defects that impair lymphocyte cytotoxicity can predispose to severe viral infection that normally remains subclinical..

Bi-Allelic Mutations in STXBP2 Reveal a Complementary Role for STXBP1 in Cytotoxic Lymphocyte Killing

Jamie A Lopez, Tahereh Noori, Adrian Minson, Lu Li Jovanoska, Kevin Thia, Michael S Hildebrand, Hedieh Akhlaghi, Phillip K Darcy, Michael H Kershaw, Natasha J Brown, Andrew Grigg, Joseph A Trapani, Ilia Voskoboinik

2018-03-15

The ability of cytotoxic lymphocytes (CL) to eliminate virus-infected or cancerous target cells through the granule exocytosis dea..

Adaptive reprogramming of NK cells in X-linked lymphoproliferative syndrome

Stephen Opat, Anna C Hearps, Kevin Thia, Agnes Yuen, Ben Rogers, Mkunde Chachage, Gregory Moore, Jake Shortt, Georgina Ryland, Piers Blombery, Anthony P Schwarer, Tahereh Noori, Joseph A Trapani, Anthony Jaworowski, Ilia Voskoboinik

2018-02-08

Real-time visualization of perforin nanopore assembly

Carl Leung, Adrian W Hodel, Amelia J Brennan, Natalya Lukoyanova, Sharon Tran, Colin M House, Stephanie C Kondos, James C Whisstock, Michelle A Dunstone, Joseph A Trapani, Ilia Voskoboinik, Helen R Saibil, Bart W Hoogenboom

2017-05-01

Perforin is a key protein of the vertebrate immune system. Secreted by cytotoxic lymphocytes as soluble monomers, perforin can sel..