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32 Cards in this Set
- Front
- Back
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Why are AAs labeled as Ketogenic, glucogenic, or both? |
Because the carbon skeleton of glucogenic AAs contains either pyruvate, or TCA cycle intermediates which can be used to form glucose via gluconeogenesis.
Ketogenic AAs on the other hand have C skeletons that are able to form Acetoacetyl-CoA, or Acetyl- CoA, and can form Ketone bodies under starvation conditions. |
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Which two AAs are exclusively ketogenic? |
Leucine and Lysine (are losers) |
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Which AAs (that we need to know) are exclusively glucogenic? |
Alanine, Asparagine, Aspartate, glutamate, glutamine |
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Which AAs (that we need to know) are both glucogenic and ketogenic |
Isoleucine, phenylalanine, tyrosine, tryptophan |
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What are the two oxidative paths Pyruvate can take follow in the body after amino group removal of alanine. |
Pyruvate Dehydrogenase reaction: produces 2.5 ATP, +10 ATP from oxidation of Acetyl CoA
Pyruvate Carboxylase (-1 ATP), forms Oxaloacetate. |
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Which enzyme converts Gln--> Glu |
Glutaminase (no energy produced) |
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How many ATP Will a citrate, or Isocitrate backbone produce? |
10 ATP |
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How much ATP can be produced from Alpha-KG |
7.5 |
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how much ATP can be produced from Succinyl CoA? |
5 ATP equivs |
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how much ATP can be produced from succinate |
4 ATP equivs |
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How much ATP can be produced from Fumarate or Malate? |
2.5 ATP |
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How much ATP can be produced from OxaloAcetate? |
0 ATP |
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What is the ATP Cost per Nitrogen in the urea cycle (including the gain of ATP from Fumarate, but not including the ATP from OX deamination) |
-1.5 ATP per nitrogen. -2 ATP for Argininosuccinate synthetase rxn, and -2ATP For Carbamoyl Phosphate synthetase. + 2.5 ATP per fumarate.
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How much ATP is produced from the oxidative deamination of Glu |
2.5 ATP (1 NADH) Glu DHase. |
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Which enzyme catalyzes the conversion of Asparagine--> Aspartate |
Asparaginase |
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How many H20s get produced from TCA cycle, not including the H20 Produced from oxidizing NADH and FADH2 at the ETC? |
-2 H20s. 1 H20 is added during the production of citrate, and another H20 is used to form Malate from fumarate |
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How many H20s get produced from TCA cycle, including the H20 Produced from oxidizing NADH and FADH2 at the ETC? |
+2 H20s. 2 H20s are invested in the Kreb cycle, one in the production of citrate, and the other in the production of OAA. 4 are regenerated from the oxidation of 3 NADH and 1 FADH2 at the ETC. |
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What is required to produce Tyrosine from Phenylalanine? |
NADH + O2 + the CoFactor THB (Tetrahydrobiopterin) |
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Why is tyrosine considered conditionally essential? |
Because Phe is essential, and we need Phe to make Tyr. If we don't have Phenylalanine hydroxylase, Tyr would be essential |
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Which enzyme uses THB as a cofactor and converts Phenylalanine into Tyrosine? |
Phenylalanine hydroxylase. |
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What is the condition called if Phenylalanine hydroxylase doesn't work, and what are the symptoms/effects. How should this be treated? |
PKU (phenylketonuria). Elevated levels of Phe are found in the blood. Tyrosine is a precursor for melanin, so they often have fair skin, and light hair. Also, the BBB allows in neutral amino acids through neutrally charged transporters. Therefore, if increased amounts of Phe are in the blood, there will be much more Phe in the brain than other AAs, cause there to be brain development issues. Low protein diet, especially low amounts of Phe. |
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People with high levels of Phe, often because of of PKU use an alternate metabolic pathway for Phe. In this pathways they produce______ and it can be detected in urine using ________, which will produce a _______ colour |
Phenyl Pyruvate. FeCl3 , green |
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What type of enzyme produced Phenylpyruvate from Phenylalanine? |
Aminotransferase |
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What is aspartame? |
It is a modified dipeptide, consisting of Aspartate and Phenylalanine |
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Why can aspartame be dangerous for people with PKU? |
Because it has a phenylalanine constituent, which cannot be digested. |
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What is the product formed from the 2 step catabolism of Tyrosine? |
Homogentisate. |
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What enzyme is deficient in people with Alkaptonuria? What are the symptoms? |
Homogentisate 1,2 -dioxygenase. elevated levels of homogentisate are excreted in the urine, and oxidation of homogentisate produces black urine. (homogentisate will be oxidized if it is present in large quantities). Oxidized homogentisate will also blacken the cartilage in the ear, and can darken the eye. |
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What are the final Carbon skeleton end products of homogentisate catabolism? is tyrosine and phenylalanine Ketogenic, glucogenic, or both? |
Fumarate, and acetoacetate. it is both glucogenic and ketogenic. Fumarate can be converted into OAA, which can undergo gluconeogenesis, and Acetoacetate is a ketone body, which can also be reduced to form B-D-hydroxybutyrate |
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What are the Carbon skeleton end products of tryptophan (11Cs) metabolism? |
Pyruvate (3Cs) and acetoacetyl-CoA (4Cs) |
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What are the Carbon skeleton end products of Leucine (6Cs) metabolism? |
Acetyl-CoA (2Cs) and Acetoacetyl CoA (4Cs) |
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What are the Carbon skeleton end products of Lysine (5Cs) metabolism? |
AcetoAcetyl CoA (4Cs) |
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What are the Carbon skeleton end products of isoLeucine (6Cs) metabolism? |
Acetyl Coa (2Cs) and Propionyl-CoA (3Cs, gets converted into Succinate) |
