sub:provenance { pmid:2022752dcterms:title "Maple syrup urine disease. Complete defect of the E1 beta subunit of the branched chain alpha-ketoacid dehydrogenase complex due to a deletion of an 11-bp repeat sequence which encodes a mitochondrial targeting leader peptide in a family with the disease" . sub:assertionhas-source:pmid:2022752 . }