PARL belongs to a family of evolutionary conserved intramembrane cleaving proteases, but it is the only member present in mitochondria. PARL has been implicated in diverse processes -- and has even been linked to Parkinson's disease -- but its exact role in health and disease remains unclear.
Research from the lab of prof. Bart De Strooper (VIB-KU Leuven) now shows that PARL plays a crucial role in the nervous system. Mice in which PARL is absent specifically in the nervous system display a striking mitochondrial phenotype, reminiscent of Leigh syndrome.
Syndrome - Disease - Neurodegeneration - Energy - Production
Leigh syndrome is a mitochondrial disease characterized by severe neurodegeneration and caused by disrupted cellular energy production. The Leuven researchers found that mice without PARL, whether through a genetic deletion in all cells or only in cells of the nervous system, show similar symptoms as patients with Leigh syndrome.
"We saw a striking neurodegeneration in our PARL mouse model, which turned out not to be due to programmed cell death or apoptosis, but due to necrosis (a type of cell death that doesn't follow the apoptotic signal transduction pathway)," says Dr. Marco Spinazzi, who digged further into the mechanisms...
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