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  -