TY - T1 - Autophagy-lysosomal defect in human CADASIL vascular smooth muscle cells SN - / UR - http://hdl.handle.net/10138/307690 T3 - A1 - Hanemaaijer, Evelyn S.; Panahi, Mahmod; Swaddiwudhipong, Nol; Tikka, Saara; Winblad, Bengt; Viitanen, Matti; Piras, Antonio; Behbahani, Homira A2 - PB - Y1 - 2018 LA - eng AB - Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a familial progressive degenerative disorder and is caused by mutations in NOTCH3 gene. Previous study reported that mutant NOTCH3 is more prone to form aggregates than wild-type NOTCH3 and the mutant aggregates are resistant to degradation. We hypothesized that aggregation or accumulation of NOTCH3 could be due to impaired lysosomal-autophagy machinery in VSMC. Here, we investigated the possi... VO - IS - SP - OP - KW - VSMC; LC3; Lysosomes; CADASIL; Autophagy; AUTOSOMAL-DOMINANT ARTERIOPATHY; SUBCORTICAL INFARCTS; NOTCH3 MUTATIONS; INHIBITION; EXPRESSION; UBIQUITIN; DEGRADATION; CHLOROQUINE; P62/SQSTM1; ACTIVATION; 3111 Biomedicine; 1182 Biochemistry, cell and molecular biology N1 - PP - ER -