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(A) In infants with classical MSUD, BCKDH activity is absent and urine and insensible losses of leucine are negligible. Thus, leucine tolerance naturally traces the balance between protein degradation and synthesis. (B) Protein accretion is energetically costly, illustrated by the close correlation between leucine tolerance (x-axis) and energy demand (y-axis) during the first 4 years of life (rs = 0.9, p < 0.0001).
