P034 Effects of a tripeptidyl peptidase-1 deficiency on the mitochondrial population of human fibroblasts Guillaume VAN BEERSEL(1) Stéphane DEMINE(1) Isabelle HAMER(2) Michel JADOT(2) and Thierry ARNOULD(1) (1) URBC, Unité de Recherche en Biochimie Cellulaire, Faculty of Sciences (2) URPhyM, Unité de Recherche en Physiologie Moléculaire, Laboratoire de Chimie Physiologique, Faculty of Medicine, Facultés Universitaires Notre-Dame de la Paix, Rue de Bruxelles, 61, 5000 Namur, Belgium Late Infantile Ceroid Lipofuscinosis (LINCL) is a neurodegenerative disorder resulting from a deficiency of the tripeptidyl peptidase-1 (TPP-1) leading to an accumulation of autofluorescent ceroid lipopigment enclosed within lysosomal structures. Knowledge about the molecular mechanisms leading to the numerous pathophysiological consequences of LINCL and cell responses to this lysosomal storage disorder remain largely unknown, but they might affect the function of other organelles such as ER and mitochondria. In this study, we investigated the putative effects of the TPP-1 deficiency on the mitochondrial population of human fibroblasts. We show that neither the abundance of mitochondria nor the mitochondrial membrane potential is modified in TPP-1 deficient cells. However, the morphology of this organelle is changed as the mitochondrial network seems to be more fragmented. This could be explained by the increase in the mitochondrial translocation of DRP-1 (Dynamin Related Protein-1). This fragmentation is accompanied by a decrease in the mitochondrial calcium buffering capacity in response to an increase in cytosolic calcium concentration.