Primary fibroblasts available from patients clinically affected by:
- Parkinson Disease (PD)
- Leber’s Hereditary Optic Neuropathy (LHON)
- Friedreich Ataxia (FRDA)
Skin fibroblasts present a system with defined mutations and the cumulative cellular damage of the patients and can thus serve as additional model to other cell systems as primary neurons or neuroblastoma cell lines.
To mimic disease related lesions of Parkinson‘s Disease, BSO (DL-buthionine-SR-sulfoximine) is applied for 48h to induce a toxic insult in PD cells. Cells are treated with or without Idebenone and cell viability is determined by MTT assay. Other possible toxic insults are available as MPP+, H2O2, Abeta1-42, 6-OHDA. Evaluation assays may range from cell viability to apoptosis assays or Mitochondrial related assays (activity, distribution, shape, membrane depolarization,…).
Figure: Effects of Idebenone on BSO induced neurotoxicity in human PD Fibroblasts.