Abstract:AbstractBackground- Dysregulation of branched chain amino acid (BCAA) catabolism caused by mutations in the metabolizing enzyme-branched chain alpha-keto acid dehydrogenase (BCKDH) leads to the fatal newborn disorder Maple Syrup Urine Disease (MSUD) and is linked to chronic diseases such as type 2 diabetes. Results- Here we show that a MSUD mouse model with severely reduced BCKDH activity exhibits glucose intolerance, alter… Show more
Set email alert for when this publication receives citations?
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.