Human Biochemistry, Volume 1 - Murray R. 1993

Metabolism of Proteins and Amino Acids
Conversion of Amino Acids into Specialized Products
β-Alanine

Free ß-Alanine is present in Tissues in small amounts; a significantly larger quantity of it is found as part of ß-alanyl dipeptides (see below) and coenzyme A (see Fig. 17.6).

Biosynthesis

In microorganisms, ß-alanine is formed via a-decarboxylation of aspartate; in mammalian tissues, it is primarily a product of the Catabolism of uracil (see Fig. 35.17), carnosine, and anserine (Fig. 32.3).

Catabolism

The catabolism of ß-alanine in mammalian tissues involves its Transamination to yield malonate semialdehyde, which is subsequently oxidized to acetate and then to СО2.

Hyper-ß-alaninemia

This rare metabolic disorder is characterized by elevated levels of free ß-alanine in plasma, CEREBROSPINAL FLUID, and urine, as well as in the Tissues of the Brain, Kidneys, Liver, and Skeletal Muscle. The concentrations of taurine and ß-aminoisobutyrate are also increased.



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