Abbreviations
The abbreviations used are: MSUD, Maple Syrup Urine Disease; BCKDH, branched chain ketoacid dehydrogenase; BCAA, branched chain amino acids; ENU, N-ethyl-N-nitrosourea; BCAT, branched chain aminotransferase; ES cell, embryonic stem cell; TRE, tetracycline responsive element; MEF, mouse embryonic fibroblast.