Our latest paper is out!

In collaboration with the Frizzell lab at the University of South Carolina we have published the article titled:

“Succination is increased on select proteins in the brainstem of the Ndufs4 knockout mouse, a model of Leigh syndrome.” 

in the journal Molecular and Cellular Proteomics.

In this paper, we have been able to identify that the metabolic deficits mediated by mitochondrial dysfunction (we used our Leigh Syndrome mice for this study) lead to permanent modifications in discrete proteins in affected neurons.

These modifications (called succination) have been shown to impair the activity of proteins in other studies, therefore we believe these mitochondrial proteins will also lose function in our model. Interestingly, our study shows that these modifications are only observed in cells residing in areas affected by the pathology, further enhancing the idea that they may mediate the selective damage observed in mitochondrial disease.

The main proteins observed to be altered, VDAC1 and VDAC2, are key factors controlling transport of ions and molecules inside the mitochondria, so these results open a new and interesting line of research in the lab in the overarching goal of finding a cure for mito disease.