Biophysical impact of sphingosine and other abnormal lipid accumulation in Niemann-Pick disease type C cell models

Niemann-Pick disease type C (NPC) is a complex and rare pathology, which is mainly associated to mutations in the NPC1 gene. This disease is phenotypically characterized by the abnormal accumulation of multiple lipid species in the acidic compartments of the cell. Due to the complexity of stored mat...

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Veröffentlicht in:	Biochimica et biophysica acta. Molecular and cell biology of lipids 2021-08, Vol.1866 (8), p.158944, Article 158944
Hauptverfasser:	Carreira, Ana C., Pokorna, Sarka, Ventura, Ana E., Walker, Mathew W., Futerman, Anthony H., Lloyd-Evans, Emyr, de Almeida, Rodrigo F.M., Silva, Liana C.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Cell Membrane - metabolism CHO Cells Cholesterol Cholesterol - metabolism Cricetinae Cricetulus Humans Lipid domains Lipid Metabolism - drug effects Lysosomal storage diseases Lysosomes - metabolism Membrane fluidity Membrane Fluidity - drug effects Models, Biological Niemann-Pick C1 Protein Niemann-Pick disease type C Niemann-Pick Disease, Type C - genetics Niemann-Pick Disease, Type C - metabolism Niemann-Pick Disease, Type C - pathology Sphingosine Sphingosine - analogs & derivatives Sphingosine - metabolism
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