Bron

Chuang D.T., Ku L.S. & Cox R.P. (1982). Thiamin-responsive maple-syrup disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate. Proc. Natl. Acad. Sci. USA 79: 3300-3304.