Degradation
Degradation of branched-chain amino acids involves the branched-chain alpha-keto acid dehydrogenase complex (BCKDH). A deficiency of this complex leads to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products in the blood and urine, giving the condition the name maple syrup urine disease.
The BCKDH complex converts branched-chain amino acids into Acyl-CoA derivatives, which after subsequent reactions are converted either into acetyl-CoA or succinyl-CoA that enter the citric acid cycle.
Enzymes involved are branched chain aminotransferase and 3-methyl-2-oxobutanoate dehydrogenase.
Read more about this topic: Branched-chain Amino Acid
Famous quotes containing the word degradation:
“Why, since man and woman were created for each other, had He made their desires so dissimilar? Why should one class of women be able to dwell in luxurious seclusion from the trials of life, while another class performed their loathsome tasks? Surely His wisdom had not decreed that one set of women should live in degradation and in the end should perish that others might live in security, preserve their frappeed chastity, and in the end be saved.”
—Madeleine [Blair], U.S. prostitute and “madam.” Madeleine, ch. 10 (1919)
“We found nothing grand in the history of the Jews nor in the morals inculcated in the Pentateuch.... I know of no other books that so fully teach the subjection and degradation of woman.”
—Elizabeth Cady Stanton (1815–1902)
“History is nothing but a procession of false Absolutes, a series of temples raised to pretexts, a degradation of the mind before the Improbable.”
—E.M. Cioran (b. 1911)