Fig 4. AGAT-d versus GAMT-d CK reaction rate evaluation. The angular coefficient (b value) of the fitting lines is an index of the CK system efficacy (b [pt 1] = 0.16 and b [pt 5] = 1.04). The CK system is down-regulated in GAMT-d (pt 1) with respect to AGAT-d (pt 5) because PCr remains low even when tCr availability is almost 90% of normal. These measures were recorded when patient 1 was treated only with Cr.