Impaired growth and neurological abnormalities in branched-chain alpha-keto acid dehydrogenase kinase-deficient mice.

@article{Joshi2006ImpairedGA,
  title={Impaired growth and neurological abnormalities in branched-chain alpha-keto acid dehydrogenase kinase-deficient mice.},
  author={Mandar A Joshi and Nam Ho Jeoung and Mariko Obayashi and Eyas M. Hattab and Eric G Brocken and Edward A. Liechty and Michael J. Kubek and Krishna M. Vattem and Ronald C Wek and Robert A. Harris},
  journal={The Biochemical journal},
  year={2006},
  volume={400 1},
  pages={153-62}
}
The BCKDH (branched-chain alpha-keto acid dehydrogenase complex) catalyses the rate-limiting step in the oxidation of BCAAs (branched-chain amino acids). Activity of the complex is regulated by a specific kinase, BDK (BCKDH kinase), which causes inactivation, and a phosphatase, BDP (BCKDH phosphatase), which causes activation. In the present study, the effect of the disruption of the BDK gene on growth and development of mice was investigated. BCKDH activity was much greater in most tissues of… CONTINUE READING

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