Amino Acid Things

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Across
  1. 1. An enzyme involved in degradation (transamination followed by oxidative decarboxylation)of branched amino acids; a deficiency causes maple-syrup urine disease
  2. 3. This 4-carbon compound is cleaved off by argininosuccinate lyase to form arginine
  3. 7. A cofactor that helps convert Phe to Tyr
  4. 8. The product of the first reaction of the urea cycle is combined with ornithine to make
  5. 10. A term used to describe amino acids whose carbon skeletons can be used to synthesize glucose
  6. 11. In the first step of the urea cycle, _____ phosphate is formed from ammonia + CO2 + ATP by _____ phosphate synthetase I
  7. 14. The only 2 exclusively ketogenic amino acids are Lysine and ______
  8. 15. Urea cycle deficiency defects have been found in each of the ____ (number) enzymes of the urea cycle
  9. 16. During starvation, this organ can use ketone bodies for fuel, but not fatty acids
  10. 18. This amino acid (along with aspartate)provides one of the amino groups used to synthesize urea
  11. 20. Citrulene is transported into the cytoplasm, where it combines with aspartate, forming argininosuccinate in a reaction catalyzed by argininosuccinate _______
  12. 21. A defect in _____ transcarbomylase (OTC)would result in low blood citrulline AND low blood arginine
  13. 22. The final step of the urea cycle is the removal of ____ from arginine, which is carried out by arginase. Ornithine is regenerated, which can be transported to the mitochondria to start the cycle anew.
Down
  1. 2. Dopamine, epinephrine, and norepinephrine are examples of
  2. 4. A nauseous patient with a urea cycle disorder who is unable to eat any food (fasting) may have elevated _____ levels
  3. 5. The enzyme arginase is limited to this organ
  4. 6. Defective Argininosuccinate lyase would result in high blood citrulline and low blood ______ (amino acid)
  5. 9. In PKU, phenylalanine is transaminated to phenylpyruvate instead of being turned into _____, which inhibits synthesis of melanin and catecholamines
  6. 12. Glutamate is the only amino acid that can undergo rapid oxidative _____ of the main chain amino group to release ammonia
  7. 13. A term used to describe amino acids whose carbon skeletons can be used to form ketone bodies
  8. 15. AcetylcoA and acetoacetate can be used to synthesize short chain ____ acids and ketone bodies
  9. 17. The first two steps of the urea cycle are carried out in the mitochondrial matrix, while the rest occurs in the _____
  10. 19. Detection of Alanine aminotransferase and Aspartate aminotransferase in the blood can be an important diagnostic tool for disease of this organ