GeneticsJournal of Inherited Metabolic DiseasePeer-reviewed
Contribution of brain intrinsic branched-chain amino acid metabolismin a novel mouse model of maple syrup urine disease
Amanda Kuhs, Laura Ohl, Tegan Thurston…·Rebecca C. Ahrens‐Nicklas
This study develops a brain-specific MSUD mouse model to investigate the role of brain-intrinsic BCAA metabolism in chronic neurologic disease, finding elevated BCAA levels and functional deficits.
6 citations