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Catabolism of Amino Acids
EVMS Biochem catabolism of amino acids
59
Biochemistry
Graduate
04/16/2011

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Term
glucogenic
Definition
if amino acid carbon skeletons can be used to synthesize glucose they are known as
alanine - pyruvate
aspartate - OAA
Term
ketogenic
Definition
Amino acids are, if their carbon skeletons make acetyl-CoA rather than substrates for gluconeogenesis, leucine and lysine
Term
glucogenic and ketogenic
Definition
some amino acids are, one portions of their carbon skeleton can make glucose while metabolism of other carbons make acetyl-CoA
Term
Leucine, Lysine and Large hydrophobic amino acids
Definition
the ketogenic amino acids, the nmenomic of the three L's
Term
1. Phenylalanine
2. Tyrosine
3. Tryptophan
4. Isoleucine
Definition
The 4 large hydrophobic AAs that are both ketogenic and glucogenic
Term
Glutamine, histidine, proline, arginine
Definition
4 AAs that are broken down to glucose via glutamate
Term
histidine
Definition
cannot be synthesized in humans
Term
liver
Definition
the full pathway of histidine degradation is only in the
Term
histidinase
Definition
the enzyme that deaminates histidine to urocanate
Term
histidinase
Definition
also found in the skin, so urocanate found in sweat
Term
FIGLU (N-formiminoglutamate)
Definition
in histidine degradation, urocanate forms
Term
N5-formino-FH4
Definition
FIGLU releases glutamate and transfers a formimino group to cofactor tetrahydrofolate in histidine breakdown to produce
Term
N5,N10 methylene FH4 and N10 Formyl FH4
Definition
deamination of N5-formino-FH4 produces in histidine degradation
Term
N5, N10 methylene-FH4 and N5-formyl-FH4
Definition
provide one carbon groups for nucleotide synthesis
Term
niacin
Definition
one pathway of tryptophan catabolism, dietary tryptophan can provide som of the required
Term
niacin
Definition
is the precursor for the nicotinamide component of NAD and NADP, a B vitamin and essential in the diet
Term
Maple Syrup Urine Disease
Definition
inborn error in the catabolism of branched chain amino acids (leucine, isoleucine, and valine, branched chain AAs found in urine
Term
a-ketoacid dehydrogenase complex
Definition
defect found in Maple Syrup urine disease, first enzyme after transamination by a ketoacid decarboxylase
Term
branched-chain amino acid transaminase
Definition
first enzyme in the oxidation of branched chain amino acids to their a-keto acids
Term
a-ketoacid dehydrogenase
Definition
second step in oxidation of branched chain amino acids, removes CO2, forms CoA intermediates
Term
first subunit
Definition
subunit of a-ketoacid dehydrogenase that catalyzes a decarboxylase reaction and utilizes thiamine as cofactor, simulare to pyruvate and aketoglutarate dehydrogenase
Term
E3 subunit
Definition
deficiency of this subunit in a-ketoacid dehydrogenas is more severe because this subunit is a component of the other two dehydrogenase complexes
Term
thiamine
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
Term
HMG-CoA lyase deficiency
Definition
rare inborn error of leucine metabolism
Term
tansamination and oxidative decarboxylation
Definition
two reactions that breaks down leucine to isovaleryl-CoA
Term
oxidation and carboxylation
Definition
two reactions that take isovaleryl-CoA and produce HMG-CoA
Term
HMG-CoA lyase
Definition
cleave HMG-CoA into acetoacetic acid and acetyl-CoA, only an intermediate in leucine catabolism, is required for both leucine catabolism and ketogenesis
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
Definition
4 symptoms of HMG-CoA lyase deficiency
Term
phenylalanine hydroxylase
Definition
enzyme for the main pathway of phenylalanine metabolism, converts it to tyrosine, BH4 is cofactor
Term
PKU (phenylketonuria)
Definition
deficiency in phenylalanine hydroxylase, increased plasma phenylalanine, excretion of phenylketones, shortage of intracellular tyrosine which causes under-pigmentation of skin and eyes
Term
tyrosine aminotransferase
Definition
catalyzes the transamination of tyrosin to hydroxyphenylpyruvate, the amino goes to glutamate
Term
fumarate, and acetoacetate
Definition
final products of phenylalanine catabolism
Term
tyrosinemia or alcaptonuria
Definition
blocks downstream from tyrosine in phenylalanine catabolism can cause
Term
transamination of Phe
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
Term
phenylketones
Definition
product of alternative phenylalanine catabolism, is excreted in urine
Term
PKU heterozygotes
Definition
appear to be protectied against a mycotoxin produced by molds infesting stored grains and other food
Term
original Guthrie test
Definition
Test, Phe permits growth of bacillus subtilis in the presence of the inhibitor B-2-thienylalanine
Term
fluorometric or mass spec assays
Definition
labs now use, to screen dried blood for multiple congenital problems
Term
BH4
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
Term
variant PkU
Definition
impaired BH3 metabolism
Term
1. phenylalanine hydroxylase
2. hydroxylation of tryptophan to 5-hydroxytryptophan
3. hydroxylation of tyrosine to L-dopa
Definition
3 roles of BH4 cofactor
Term
dihydropteridine reductase
Definition
individuals deficient in this cannot recycle the BH2 cofactor, has impaired synthesis of neurotransmitters
Term
dihydropteridine synthesis
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
Term
branch chain amino acids
Definition
muscle selectively oxidizes
Term
alanine and glutamine
Definition
are synthesized and exported from muscle during fasting or illness
Term
alanine
Definition
transfers nitrogen to the liver for urea synthesis, the carbon skeleton is used for gluconeogenesis
Term
Glutamine
Definition
provides NH4 for kidney to buffer excretion of organic acids. It also serves as a feul for the kidney
Term
the alanine cycle
Definition
provides glucose to muscle for synthesis of alanine and glutamine
Term
glutamine synthetase
Definition
in muscle induced by glucocorticoids, enzyme needed during fasting and muscle wasting
Term
fasting and high protein intakes
Definition
in liver the enzymes of amino acid degradation and of the urea cycle are induced by
Term
plasma ALT and AST
Definition
inicators of liver damage
Term
phenylalanine hydroxylase
Definition
PKU enzyme, is a liver enzyme
Term
glutamine
Definition
renal tubule cells oxidize
Term
NH4
Definition
serves to buffer the urine, need more during fasting due to metabolic acidosis
Term
a-ketoglutarate
Definition
can be used for gluconeogenesis by the renal cortex via the TCA cycle
Term
Glutamine
Definition
a significant source of CO2 at all times in the kidney, and increases during acidosis
Term
Warburg effect
Definition
in transformed cells, major use of glucose produces lactate even when oxygen is present
Term
pentose phosphate pathway
Definition
active in transformed cells for more nucleotide synthesis and rapid growth
Term
Glutamine
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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