Sundararajan Venkatesh

Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy.

Protein quality control at the interface of endoplasmic reticulum and mitochondria by Lon protease.

Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo.