Reactome | PPM1K dephosphorylates p-BCKDH

PPM1K dephosphorylates p-BCKDH

Stable Identifier

R-HSA-5693153

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

H2O + O-phospho-L-seryl-(3-methyl-2-oxobutanoate dehydrogenase) => L-seryl-(3-methyl-2-oxobutanoate dehydrogenase) + phosphate

ReviewStatus

5/5

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The branched-chain alpha-ketoacid dehydrogenase (BCKDH) complex occupies a strategic point in the branched-chain amino acid (BCAA) catabolic pathway, and careful regulation of its activity is essential for correct BCAA metabolism. The overall activity of the BCKDH complex is controlled by the phosphorylation (inactivation)/dephosphorylation (activation) cycle. Mitochondrial protein phosphatase 1K (PPM1K) dephosphorylates the E1 beta subunit of BCKDH thereby regaining its active state. PPM1K requires Mn2+ as a cofactor for phosphatase activity (Wynn et al. 2012).

Literature References

PubMed ID	Title	Journal	Year
22291014	Structural and biochemical characterization of human mitochondrial branched-chain α-ketoacid dehydrogenase phosphatase Li, J, Chuang, DT, Chuang, JL, Wynn, RM, Brautigam, CA	J. Biol. Chem.	2012