Autophagy-lysosomal defect in human CADASIL vascular smooth muscle cells

•CADASIL is an inherited disease of cerebral vascular cells.•Increased intracellular domain of NOTCH3 in VSMCR133C.•Increased amount of lysosomes co-localized with NOTCH3 in VSMCR133C.•Decreased co-localization of NOTCH3 with LC3 and LAMP2 indicating a defect in the autophagy-lysosomal pathway.•Dysr...

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Veröffentlicht in:	European journal of cell biology 2018-11, Vol.97 (8), p.557-567
Hauptverfasser:	Hanemaaijer, Evelyn S., Panahi, Mahmod, Swaddiwudhipong, Nol, Tikka, Saara, Winblad, Bengt, Viitanen, Matti, Piras, Antonio, Behbahani, Homira
Format:	Artikel
Sprache:	eng
Schlagworte:	Autophagosomes - drug effects Autophagosomes - metabolism Autophagy Autophagy - drug effects CADASIL CADASIL - pathology Chloroquine - pharmacology Humans LC3 Lysosomes Lysosomes - drug effects Lysosomes - pathology MAP Kinase Signaling System - drug effects Medicin och hälsovetenskap Microtubule-Associated Proteins - metabolism Models, Biological Muscle, Smooth, Vascular - pathology Myocytes, Smooth Muscle - drug effects Myocytes, Smooth Muscle - pathology Phosphorylation - drug effects Proteolysis - drug effects Receptor, Notch3 - metabolism Sequestosome-1 Protein - metabolism VSMC
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