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isoleucine on Encyclopedia.com (Site not responding. Last check: 2007-11-06) |
 | | In a rare, inherited disorder called maple syrup urine disease, a nonfunctional enzyme in the common pathway of isoleucine, leucine, and valine degradation causes the buildup of certain metabolites in the urine, resulting in the characteristic odor from which the disease derives its name. |
 | | Once isoleucine is incorporated into protein, it contributes to the structure of protein by the tendency of its side chain (composed only of carbon and hydrogen) to seek an environment consisting of similar side chains, like those of leucine, valine, tryptophan, and phenylalanine, and to exclude water. |
 | | Isoleucine was isolated from beet sugar molasses in 1904. |
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