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45 The second step is an irreversible rate-limiting reaction catalyzed by branched-chain -keto acid dehydrogenase (BCKDH), which is phosphorylated and inactivated by the specific kinase BCKDH kinase (BCKDK) and dephosphorylated and activated by Protein phosphatase 1 K (PPM1K)
This finding aligns with current AAFP clinical guidance recommending oral therapy as a first-line option for most patients
& Shokat, K
