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