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BioChem 39 AminoAcid

LECOM BioChem Ch 39 Synthesis and Degredation of Amino Acids

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