Lowered Concentrations of Branched-Chain Amino Acids Result in Impaired Growth and Neurological Problems: Insights from a Branched-Chain α-Keto Acid Dehydrogenase Complex Kinase-Deficient Mouse Model

Author: Watford, Malcolm

Source: Nutrition Reviews, Volume 65, Number 4, April 2007 , pp. 167-172(6)

Publisher: International Life Sciences Institute

Key:

- Free Content

- New Content

- Subscribed Content

- Free Trial Content

Abstract:

Excess circulating levels of branched-chain amino acids (BCAA), as seen in maple syrup urine disease, result in severe neuropathology. A new mouse model, deficient in the kinase that controls BCAA catabolism, shows that very low circulating levels of BCAA are also associated with neuropathology, including the development of epileptic seizures. These mice clearly demonstrate the need to control essential amino acid levels within both upper and lower limits.

Keywords: ALPHA-KETO ACID DEHYDROGENASE; BRANCHED-CHAIN AMINO ACIDS; BRANCHED-CHAIN KETO ACIDS; MAPLE SYRUP URINE DISEASE

Document Type: Review article

DOI: 10.1301/nr.2007.apr.167-172

Your trusted access to this article has expired.

The full text electronic article is available for purchase. You will be able to download the full text electronic article after payment.

$20.00 plus tax Refund Policy