Term
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Definition
if amino acid carbon skeletons can be used to synthesize glucose they are known as
alanine - pyruvate
aspartate - OAA |
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Term
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Definition
| Amino acids are, if their carbon skeletons make acetyl-CoA rather than substrates for gluconeogenesis, leucine and lysine |
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Term
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Definition
| some amino acids are, one portions of their carbon skeleton can make glucose while metabolism of other carbons make acetyl-CoA |
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Term
| Leucine, Lysine and Large hydrophobic amino acids |
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Definition
| the ketogenic amino acids, the nmenomic of the three L's |
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Term
1. Phenylalanine
2. Tyrosine
3. Tryptophan
4. Isoleucine |
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Definition
| The 4 large hydrophobic AAs that are both ketogenic and glucogenic |
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Term
| Glutamine, histidine, proline, arginine |
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Definition
| 4 AAs that are broken down to glucose via glutamate |
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Term
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Definition
| cannot be synthesized in humans |
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Term
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Definition
| the full pathway of histidine degradation is only in the |
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Term
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Definition
| the enzyme that deaminates histidine to urocanate |
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Term
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Definition
| also found in the skin, so urocanate found in sweat |
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Term
| FIGLU (N-formiminoglutamate) |
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Definition
| in histidine degradation, urocanate forms |
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Term
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Definition
| FIGLU releases glutamate and transfers a formimino group to cofactor tetrahydrofolate in histidine breakdown to produce |
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Term
| N5,N10 methylene FH4 and N10 Formyl FH4 |
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Definition
| deamination of N5-formino-FH4 produces in histidine degradation |
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Term
| N5, N10 methylene-FH4 and N5-formyl-FH4 |
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Definition
| provide one carbon groups for nucleotide synthesis |
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Term
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Definition
| one pathway of tryptophan catabolism, dietary tryptophan can provide som of the required |
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Term
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Definition
| is the precursor for the nicotinamide component of NAD and NADP, a B vitamin and essential in the diet |
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Term
| Maple Syrup Urine Disease |
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Definition
| inborn error in the catabolism of branched chain amino acids (leucine, isoleucine, and valine, branched chain AAs found in urine |
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Term
| a-ketoacid dehydrogenase complex |
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Definition
| defect found in Maple Syrup urine disease, first enzyme after transamination by a ketoacid decarboxylase |
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Term
| branched-chain amino acid transaminase |
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Definition
| first enzyme in the oxidation of branched chain amino acids to their a-keto acids |
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Term
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Definition
| second step in oxidation of branched chain amino acids, removes CO2, forms CoA intermediates |
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Term
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Definition
| subunit of a-ketoacid dehydrogenase that catalyzes a decarboxylase reaction and utilizes thiamine as cofactor, simulare to pyruvate and aketoglutarate dehydrogenase |
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Term
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Definition
| deficiency of this subunit in a-ketoacid dehydrogenas is more severe because this subunit is a component of the other two dehydrogenase complexes |
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Term
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Definition
| some patients of maple syrup urine disease are responsive to high doese of this, due to the mutant enzyme having a lower affinity for the cofactor |
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Term
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Definition
| rare inborn error of leucine metabolism |
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Term
| tansamination and oxidative decarboxylation |
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Definition
| two reactions that breaks down leucine to isovaleryl-CoA |
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Term
| oxidation and carboxylation |
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Definition
| two reactions that take isovaleryl-CoA and produce HMG-CoA |
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Term
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Definition
| cleave HMG-CoA into acetoacetic acid and acetyl-CoA, only an intermediate in leucine catabolism, is required for both leucine catabolism and ketogenesis |
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Term
1. metabolic acidosis and organic aciduria
2. fasting hypoglycemia without ketosis
3. hepatomegaly with diffuse accumulation of lipid droplets
4. lethargy or coma and apnea |
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Definition
| 4 symptoms of HMG-CoA lyase deficiency |
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Term
| phenylalanine hydroxylase |
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Definition
| enzyme for the main pathway of phenylalanine metabolism, converts it to tyrosine, BH4 is cofactor |
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Term
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Definition
| deficiency in phenylalanine hydroxylase, increased plasma phenylalanine, excretion of phenylketones, shortage of intracellular tyrosine which causes under-pigmentation of skin and eyes |
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Term
| tyrosine aminotransferase |
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Definition
| catalyzes the transamination of tyrosin to hydroxyphenylpyruvate, the amino goes to glutamate |
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Term
| fumarate, and acetoacetate |
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Definition
| final products of phenylalanine catabolism |
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Term
| tyrosinemia or alcaptonuria |
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Definition
| blocks downstream from tyrosine in phenylalanine catabolism can cause |
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Term
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Definition
| alternative pathway for phenylalanine catabolism, works on phenylalanine, phenylpyruvate and other metabolites, has a high Km so phenylalanine levels will still be high, produces phenylketones |
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Term
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Definition
| product of alternative phenylalanine catabolism, is excreted in urine |
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Term
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Definition
| appear to be protectied against a mycotoxin produced by molds infesting stored grains and other food |
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Term
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Definition
| Test, Phe permits growth of bacillus subtilis in the presence of the inhibitor B-2-thienylalanine |
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Term
| fluorometric or mass spec assays |
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Definition
| labs now use, to screen dried blood for multiple congenital problems |
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Term
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Definition
| is synthesized from GTP, unlike other cofactors that are synthesized from vitamins, donates hydrogen atoms during hydroxylation reactions, two steps are required to regenerate |
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Term
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Definition
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Term
1. phenylalanine hydroxylase
2. hydroxylation of tryptophan to 5-hydroxytryptophan
3. hydroxylation of tyrosine to L-dopa |
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Definition
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Term
| dihydropteridine reductase |
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Definition
| individuals deficient in this cannot recycle the BH2 cofactor, has impaired synthesis of neurotransmitters |
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Term
| dihydropteridine synthesis |
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Definition
| individuals deficient in this enzyme cannot synthesize BH2 and is required as a vitamin, and lipophilic analong of BH2 may cross the blood brain barrier |
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Term
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Definition
| muscle selectively oxidizes |
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Term
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Definition
| are synthesized and exported from muscle during fasting or illness |
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Term
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Definition
| transfers nitrogen to the liver for urea synthesis, the carbon skeleton is used for gluconeogenesis |
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Term
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Definition
| provides NH4 for kidney to buffer excretion of organic acids. It also serves as a feul for the kidney |
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Term
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Definition
| provides glucose to muscle for synthesis of alanine and glutamine |
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Term
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Definition
| in muscle induced by glucocorticoids, enzyme needed during fasting and muscle wasting |
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Term
| fasting and high protein intakes |
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Definition
| in liver the enzymes of amino acid degradation and of the urea cycle are induced by |
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Term
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Definition
| inicators of liver damage |
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Term
| phenylalanine hydroxylase |
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Definition
| PKU enzyme, is a liver enzyme |
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Term
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Definition
| renal tubule cells oxidize |
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Term
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Definition
| serves to buffer the urine, need more during fasting due to metabolic acidosis |
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Term
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Definition
| can be used for gluconeogenesis by the renal cortex via the TCA cycle |
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Term
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Definition
| a significant source of CO2 at all times in the kidney, and increases during acidosis |
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Term
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Definition
| in transformed cells, major use of glucose produces lactate even when oxygen is present |
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Term
| pentose phosphate pathway |
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Definition
| active in transformed cells for more nucleotide synthesis and rapid growth |
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Term
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Definition
| major feul in transformed cells, aKG to OAA provides NADH for ATP synthesis, OAA exported as citrate, Acetyl CoA produces membrane lipids, Malic enzyme provides NADPH |
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