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BioChem 39 AminoAcid
LECOM BioChem Ch 39 Synthesis and Degredation of Amino Acids
| Question | Answer |
|---|---|
| Which 2 non-essential amino acids require a precursor amino acid for synthesis? | tyrosine and cysteine |
| α-ketoglutarate is the precursor for synthesis of what 4 amino acids? | glutamate, glutamine, proline, arginine |
| Oxaloacetate provides the carbon for what 2 amino acids? | asparagine and aspartate |
| What 6 amino acids produce pyruvate when they are degraded? | serine, alanine, cysteine, threonine, glycine, tryptophan |
| α-ketoglutarate is produced when this amino acid is degraded. | glutamate |
| These 4 AA are converted to glutamate then α-ketoglutarate when degraded. | Arginine, Histadine, Glutamine, Proline |
| These 2 AA are broken down into oxaloacetate | aspartate and asparagine |
| These 4 AA are broken down into Succinyl CoA when degraded | Valine, Threonine, Isoleucine, Methionine |
| Only 2 AA that are strictly ketogenic | lysine and leucine |
| 4 AA that can be degraded to Acetyl CoA | Threonine, Isoleucine, Lysine, Tryptophan |
| 3 AA that can become Acetoacetate/ketone body | Leucine, Phenylalanine, Tyrosine |
| This is the quintessential cooenzyme of amino acid metabolism | pyridoxal phosphate |
| This coenzyme is used in AA pathways to accept or donate one-carbon groups | tetrahydrofolate |
| This coenzyme is required for ring hydroxylations | tetrahyrobiopterin |
| 4 amino acids which are synthesized from glycolysis intermediates | serine, glycine, cysteine and alanine |
| Serine synthesis sites | liver and kidney |
| When serine levels fall, negative regulation of these two enzymes help produce serine | 3-phosphoglycerate dehydrogenase and phosphoserine phosphatase |
| Kidney failure due to stones is caused by deficiency of this enzyme; name of disease | transaminase of glyoxylate to glycine; PH-1, Primary oxaluria type I |
| Glycine is synthesized mostly from | serine |
| To form glycine from serine you need | FH4 and PLP with serine hydroxymethyl transferase |
| To form cysteine you need what 2 AA? | serine and methionine |
| This AA is the major gluconeogenic AA | alanine |
| B6(pyridoxine) deficiency can cause cardiovascular disease by increasing | homocysteine |
| 5 AA associated with α-ketoglutarate | glutamate, glutamine, proline, arginine, histidine |
| Glutamate is used for the synthesis of these 4 AA | glutamine, proline, arginine, ornithine |
| Glutamine is produced from what, with what enzyme? | from glutamate with glutamine synthatase (1 of 3 human enzymes that can add NH4+ to an organic molecule |
| Glutamine to glutamate is helpful when done where? | kidney to help excrete acid |
| Proline is made from what AA? | glutamate |
| Pathway of arginine from glutamate: | Glutamate-glutamate semialdehyde – ornithine->urea cycle->arginine |
| Is arginine an essential AA? | No, until periods of growth, which it is then needed |
| Is histidine an essential AA? | yes |
| Histamine degradation pathway | Histamine -> FIGLU-> C+NH4+ glutamate |
| 2 AA related to oxaloacetate | aspartate and asparagine |
| Aspartate is made from | transamination of oxaloacetate +PLP |
| Asparagine is made from | aspartate + nitrogen from glutamine by asparagine synthetase |
| Aspartate can be broken down into what 2 different end products | axaloacetate; fumarate |
| Tyrosine is formed from what AA | Phenylalanine |
| What is the fate of tyrosine | degraded to acetoacetate and fumarate |
| Does phenylalanine to fumarate produce energy? | no |
| When in phenylalanine degradation is any energy made? | after fumarate to malate to carbon for gluconeogenesis |
| 4 essential AA that are degraded to Succinyl CoA | methionine, valine, isoleucine, and threonine |
| 2 coenzymes needed to make Succinyl CoA from Propionyl CoA | Biotin and B12 |
| 4 coenzymes to make Succinyl CoA from Methionine | 2 PLP, Biotin and B12 |
| 2 coenzymes needed to regenerate Methionine from homocysteine | FH4 and B12 |
| Threonine is degraded to what 2 intermediates and with what coenzyme | ammonia and α-ketobutyrate with PLP |
| Methionine to Succinyl CoA steps (8) | Meth->homocysteine->cystathionine->α-ketobutyrate ->Propionyl CoA->D-methylmalonyl CoA ->L-methylmalonyl CoA ->Succinyl CoA |
| 3 branched-chain AA | valine, leucine, isoleucine |
| Tissue where most branched-chain AA oxidation is done | muscles |
| 2 goals of valine and isoleucine degradation | energy and replenish TCA cycle |
| PAH is the missing enzyme. What is the Disease and what accumulates? | classic PKU; phenylalanine |
| Dihydropteridine reductase is the missing enzyme. What is the Disease and what accumulates? | nonclassical PKU; phenylalanine |
| Homogentisate oxidase is the missing enzyme. What is the Disease and what accumulates? | Alcaptonuria; Homogenistic acid |
| Fumarylacetoacetate hydrolase is the missing enzyme. What is the Disease and what accumulates? | Tyrosinemia I; fumarylacetoacetate |
| Tyrosine aminotransferase is the missing enzyme. What is the Disease and what accumulates? | Tyrosinemia II; tyrosine |
| Cystationase is the missing enzyme. What is the Disease and what accumulates? | Cystithioninuria; cystathione |
| Cystathionine Beta synthase is the missing enzyme. What is the Disease and what accumulates? | Homocysteinemia; homocysteine |
| Glycine transaminase is the missing enzyme. What is the Disease and what accumulates? | Primary oxaluria type I; glyocylate |
| Branched chain alpha keto acid dehydrogenase is the missing enzyme. What is the Disease and what accumulates? | maple syrup urine disease; alpha keto acids of the branched chain amino acids |
Created by:
csheck
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