Back to Search
Start Over
Lipid profiling of parkin-mutant human skin fibroblasts.
- Source :
-
Journal of cellular physiology [J Cell Physiol] 2017 Dec; Vol. 232 (12), pp. 3540-3551. Date of Electronic Publication: 2017 Feb 10. - Publication Year :
- 2017
-
Abstract
- Parkin mutations are a major cause of early-onset Parkinson's disease (PD). The impairment of protein quality control system together with defects in mitochondria and autophagy process are consequences of the lack of parkin, which leads to neurodegeneration. Little is known about the role of lipids in these alterations of cell functions. In the present study, parkin-mutant human skin primary fibroblasts have been considered as cellular model of PD to investigate on possible lipid alterations associated with the lack of parkin protein. Dermal fibroblasts were obtained from two unrelated PD patients with different parkin mutations and their lipid compositions were compared with that of two control fibroblasts. The lipid extracts of fibroblasts have been analyzed by combined matrix-assisted laser desorption/ionization-time-of-flight mass spectrometry (MALDI-TOF/MS) and thin-layer chromatography (TLC). In parallel, we have performed direct MALDI-TOF/MS lipid analyses of intact fibroblasts by skipping lipid extraction steps. Results show that the proportions of some phospholipids and glycosphingolipids were altered in the lipid profiles of parkin-mutant fibroblasts. The detected higher level of gangliosides, phosphatidylinositol, and phosphatidylserine could be linked to dysfunction of autophagy and mitochondrial turnover; in addition, the lysophosphatidylcholine increase could represent the marker of neuroinflammatory state, a well-known component of PD.<br /> (© 2017 Wiley Periodicals, Inc.)
- Subjects :
- Biomarkers metabolism
Cells, Cultured
Chromatography, Thin Layer
Fibroblasts pathology
Genetic Predisposition to Disease
Humans
Parkinson Disease genetics
Parkinson Disease pathology
Phenotype
Primary Cell Culture
Skin pathology
Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
Fibroblasts metabolism
Glycosphingolipids metabolism
Mutation
Parkinson Disease metabolism
Phospholipids metabolism
Skin metabolism
Ubiquitin-Protein Ligases genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1097-4652
- Volume :
- 232
- Issue :
- 12
- Database :
- MEDLINE
- Journal :
- Journal of cellular physiology
- Publication Type :
- Academic Journal
- Accession number :
- 28109117
- Full Text :
- https://doi.org/10.1002/jcp.25815