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Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.
Structure. 2000 Mar 15;8(3):277-91. doi: 10.1016/s0969-2126(00)00105-2.
Structure. 2000.
PMID: 10745006
Free article.
Heterogeneity of mutations in maple syrup urine disease (MSUD): screening and identification of affected E1 alpha and E1 beta subunits of the branched-chain alpha-keto-acid dehydrogenase multienzyme complex.
Nobukuni Y, Mitsubuchi H, Hayashida Y, Ohta K, Indo Y, Ichiba Y, Endo F, Matsuda I.
Nobukuni Y, et al.
Biochim Biophys Acta. 1993 Nov 25;1225(1):64-70. doi: 10.1016/0925-4439(93)90123-i.
Biochim Biophys Acta. 1993.
PMID: 8161368
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