Article
A family with Papillon-Lefevre syndrome reveals a requirement for cathepsin C in granzyme B activation and NK cell cytolytic activity.
Blood - 1 May 2006
Meade Josephine L, de Wynter Erika A, Brett Peter, Sharif Saghira Malik, Woods C Geoffrey, Markham Alexander F, Cook Graham P
Abstract excerpt
Activation of granzyme B, a key cytolytic effector molecule of natural killer (NK) cells, requires removal of an N-terminal pro-domain. In mice, cathepsin C is required for granzyme processing and normal NK cell cytolytic function, whereas in patients with Papillon-Lefèvre syndrome (PLS), loss-of-function mutations in cathepsin C do not affect lymphokine activated killer (LAK) cell function. Here we demonstrate...
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