TY  - JOUR 
AU  - Bullón, Pedro
AU  - Varela López, Alfonso
AU  - Quiles Morales, José Luis
AU  - Cordero, Mario
PY  - 2018
UR  - http://hdl.handle.net/10481/64979
AB  - Background: Cathepsin C (CatC) is a lysosomal enzyme involved
in activation of serine proteases from immune and inflammatory
cells. Several loss-of-function mutations in the CatC gene have
been shown to be the genetic mark of Papillon-Lef evre...
LA  - eng
PB  - MOSBY-ELSEVIER
KW  - Papillon-Lefevre syndrome
KW  - Cathepsin C
KW  - Autophagy
KW  - Lysosomal permeabilization
TI  - Autophagic dysfunction in patients with papillon-lefevre syndrome is restored by recombinant cathepsin C treatment
DO  - 10.1016/j.jaci.2018.01.018
ER  -