Welcome to Lars Eide's group Mitogroup
Nuclear and mitochondrial genomes (nDNA and mtDNA, respectively) are subjected to dynamic modifications. There is a crosstalk between mitochondrial metabolism and mtDNA modifications.
Our aim is to understand how modifications on mtDNA influence/regulate mitochondrial metabolism.
Relevant diseases are:
- Maple Syrup Urine Disease (MSUD); a rare, inborn disease caused by deficient degradation of branched chain amino acids. This degradation is rate-limited by a large branched-chain keto acid dehydrogenase complex, whose core subunit is also a constituent of the “mitochondrial chromatin”.
- Pancreatic Ductal adenocarcinoma (PDAC) has reprogrammed energy metabolism towards branched-chain amino acid metabolism.
- Huntington’s disease (HD) is a neurodegenerative disorder, with strong association to mitochondrial dysfunction. We have found that nuclear DNA integrity is a peripheral biomarker that correlates with disease progression, and speculate that individual mitochondrial capacity dictates age of onset.
- Branched-chain amino acids are precursors for branched-chain fatty acids; with unknown function but present from gestation and throughout life, and positively associated with glucose homeostasis.