Overview
Investigating Calcium-induced mitochondrial dysfunction in zebrafish and iPSC models of SMA
- Principal investigator(s):
- Dr. Sorana Ciura
- Institution:
- Institut Imagine, France
- Grant:
- €90,000
- Grant Type:
- Operating Grant
- Start Year:
- 2024
- Duration:
- 2 Years
- Call number:
- 12
- Status:
- Not yet started
Dr. Sorana Ciura
Dr. Ciura is a tenured researcher and theme leader in the "Translational research for neurological disorders" lab, directed by Edor Kabashi, at the Institute Imagine of the Necker Hospital for Sick Children in Paris, France. She has graduated from University of Toronto and obtained her PhD in Neurophysiology from McGill University, Canada. Her research is centred around mechanisms of neuromuscular diseases using a combination of zebrafish models and patient derived iPSCs.
In Focus
Background
While recent years have seen the development of therapies for SMA that effectively increase SMN protein levels (the depletion of which causes the disease), many patients still experience severe symptoms, underscoring the need for complementary treatments that target multiple systems.
How will Dr. Ciura’s team do this?
To address this, Dr. Ciura and her team have developed a new zebrafish model mimicking SMA, which shows versatility for both mechanistic investigations and screening platforms to test new drugs. By studying this model, the team has found links between calcium levels, mitochondria (the cell's energy factories), and SMA symptoms.
Why is it interesting to patients?
Using both zebrafish and patient-derived cells, the team will now explore whether targeting calcium pathways could be a new strategy to treat SMA.