Lysosomes dysfunction causes mitophagy impairment in PBMCs of sporadic ALS patients
Authors/Creators
- 1. IRCCS Mondino Foundation (Italy)
- 2. IRCCS Mondino Foundation, Pavia; University of Pavia (Italy)
- 3. University of Milan (Italy)
- 4. Casa di Cura Ars Medica, Rome (Italy)
- 5. Golgi-Cenci Foundation, Abbiategrasso-MI- (Italy)
- 6. IRCCS San Matteo Foundation, Pavia (Italy)
Description
The database includes the raw data of the article “Lysosomes dysfunction causes mitophagy impairment in PBMCs of sporadic ALS patients”. Aim of this work was to investigate mitophagy pathway in PBMCs of ssporadic amyotrophic lateral sclerosis (sALS) patients and how the pathway can be tuned by using chemicals.
The database contains the data obtained by the following evaluations: a) flow cytometry for the evaluation of mitochondria health, b) western blot analysis of protein involved in mitophagy and mitochondrial dynamism, c) immunofluorescence analysis of autophagosomes and lysosomes accumulation.
We evidenced that that the presence of morphologically altered mitochondria and an inefficient turnover of damaged mitochondria in PBMCs of sALS patients relies on an impairment of the mitophagy pathway. In particular, we found that the induction of mTOR-independent autophagy pathway leads to a decrease of lysosomes level, suggesting a more sensitivity of sALS PBMCs to the effect of trehalose. Such evidences suggest that trehalose could represent an effective treatment for ALS patients.
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Related works
- Is published in
- Journal article: 10.3390/cells11081272 (DOI)