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Series GSE109228 Query DataSets for GSE109228
Status Public on Jan 16, 2019
Title Necroptosis inhibition protects from dopaminergic neuronal cell death in OPA1 mutant Parkinson’s disease patient neurons and MPTP treated mice
Organism Homo sapiens
Experiment type Expression profiling by high throughput sequencing
Summary Dysfunctions in mitochondria dynamics and metabolism are common pathological processes associated with Parkinson’s disease (PD). Recently, it was shown that an inherited form of PD and dementia is caused by new mutations in the OPA1 gene, which encodes for a key player of mitochondrial fusion and structure. iPSC-derived neural cells from these patients exhibited severe mitochondrial fragmentation, respiration impairment, ATP deficits and heightened oxidative stress. Reconstitution of normal levels of OPA1 in PD-derived neural cells normalized mitochondria morphology and function. OPA1 mutated neuronal cultures showed reduced survival in vitro. Intriguingly, selective inhibition of necroptosis effectively rescued this survival deficit. Additionally, dampening necroptosis in MPTP treated mice protected from DA neuronal cell loss. This human iPSC-based model captures both the early pathological events in OPA1 mutant neural cells and the beneficial effects of blocking necroptosis, highlighting this cell death process as a promising therapeutic target for PD.
Overall design 3 replicates for control and 3 replicates for OPA1 F38D mutant cells
Contributor(s) Broccoli V, Iannielli A, Massimino L, Bido S
Citation(s) 29466734
Submission date Jan 16, 2018
Last update date Mar 18, 2019
Contact name Luca Massimino
Phone 3389039500
Organization name Ospedale San Raffaele
Department Gastroenterology
Street address Via Olgettina, 58
City Milano
State/province MI
ZIP/Postal code 20100
Country Italy
Platforms (1)
GPL21290 Illumina HiSeq 3000 (Homo sapiens)
Samples (6)
GSM2935600 Control cells 37_1
GSM2935601 Control cells 37_2
GSM2935602 Control cells 68_1
BioProject PRJNA430219
SRA SRP131002

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Supplementary file Size Download File type/resource
GSE109228_RAW.tar 2.2 Mb (http)(custom) TAR (of TAB)
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Raw data are available in SRA
Processed data provided as supplementary file

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