Defective Autophagy, Mitochondrial Clearance and Lipophagy in Niemann-Pick Type B Lymphocytes

Niemann-Pick disease type A (NP-A) and type B (NP-B) are lysosomal storage diseases (LSDs) caused by sphingomyelin accumulation in lysosomes relying on reduced or absent acid sphingomyelinase. A considerable body of evidence suggests that lysosomal storage in many LSD impairs autophagy, resulting in...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	PloS one 2016-10, Vol.11 (10), p.e0165780-e0165780
Hauptverfasser:	Canonico, Barbara, Cesarini, Erica, Salucci, Sara, Luchetti, Francesca, Falcieri, Elisabetta, Di Sario, Gianna, Palma, Fulvio, Papa, Stefano
Format:	Artikel
Sprache:	eng
Schlagworte:	Accumulation Analysis Apoptosis Autophagy B cells B-Lymphocytes - metabolism B-Lymphocytes - ultrastructure Biology and Life Sciences Biomarkers Brain diseases Cell death Cell-Derived Microparticles - metabolism Damage accumulation Disease Endocytosis Exocytosis Extracellular Space - metabolism Extrusion Fibroblasts Flow Cytometry Genetic disorders Humans Intracellular Space - metabolism Lipid Metabolism Lipids Lymphocytes Lymphocytes B Lysosomal storage diseases Lysosomes Lysosomes - metabolism Medicine and Health Sciences Melatonin Metabolic disorders Microscopy Mitochondria Mitochondria - metabolism Mitochondria - ultrastructure Mitochondrial Degradation Neurodegeneration Niemann-Pick disease Niemann-Pick Diseases - metabolism Nocodazole Phagocytosis Phagosomes Proteins Rapamycin Research and Analysis Methods Sphingomyelin Sphingomyelin phosphodiesterase Storage
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Schreiben Sie den ersten Kommentar!