human BCKDH has two phosphorylation sites targeted by BCKDK, Ser292-alpha and Ser302-alpha, that both reside in the E1 catalytic site. Phosphorylation of Ser292-alpha alone inactivates BCKDH, while phosphorylation of Ser302-alpha appears to have no impact. Dephosphorylation of E1 is carried out by the protein phosphatase Mg2+/Mn2+ dependent 1K (PP2Cm, encoded by PPM1K), thereby activating BCKDH. Phosphorylation of BCKDH destabilizes BCAT2's interaction with BCKDH
the activity of the BCKD complex is regulated by phosphorylation and dephosphorylation catalyzed by a kinase and a phosphatase, respectively. The BCKD kinase inhibits the complex whereas the BCKD phosphatase activates it
BCKD phosphorylation by BCKD kinase (BCKDK) inactivates BCKD and cause neurocognitive dysfunction, whereas dephosphorylation by specific phosphatase restores BCKD activity
BCKD phosphorylation by BCKD kinase (BCKDK) inactivates BCKD and cause neurocognitive dysfunction, whereas dephosphorylation by specific phosphatase restores BCKD activity