A patient with maple syrup urine disease (MSUD) associated with a E1β subunit deficiency of the branced-chain α-keto acid dehydrogenase (BCKDH) complex was investigated at the molecular level. The defect responsible for the deficiency of the E1β subunit protein was identified by analysis of...
Corn Syrup (light and dark):All baking (except candy-making): honey, agave, brown rice syrup, cane syrup, maple syrup or golden syrup. Alternatively, for 1 cup light corn syrup, combine 1 cup sugar and ¼ cup hot water. For candy-making: brown rice syrup or golden syrup. Cream of ...