CORDIS Project
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This research project investigates the immune dysfunction in Barth Syndrome by examining the role of cardiolipin in regulating immune responses. It aims to understand how modifications in cardiolipin affect the activation of key immune receptors, potentially leading to new therapeutic targets for this disease.
Barth Syndrome (BTHS) is a disease characterised by reduced production of unsaturated mitochondrial cardiolipin (CL) and increased production of abnormal CL with saturated chains.
Chronic inflammation and increased vulnerability to infection are major contributors to mortality in BTHS patients.
The connection between these immune dysfunctions and CL modifications is not understood.
My research has shown that unsaturated CL inhibits caspase 4 (CASP4) and Toll-like Receptor 4 (TLR4).
Moderate CASP…
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