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Chapter 20-Amino Acid Metabolism

→ The major source of amino acids is the diet. Humans can only synthesize 11 of the 20 common amino acids. The other 9 (H I L K M F T W V) are essential. Arginine is essential only during growth. Tyr is not essential, but only because it can be readily synthesized from the essential Phe. → No special storage compartment- all are in functional proteins- last to use as energy source →Many of the amino acids can be synthesized from pyruvate,PEP (glycolysis), 3-phosphoglycerate (glycolysis) or TCA cycle intermediates (see figure 16.14) Example: Pyruvate ⇔ alanine in 1 step a-ketoglutarate ⇔ Glu →→→Arg → The amino acids are degraded by 20 different pathways that converge to just 7 metabolic intermediates (see figure 21.13). These intermediates enter the TCA cycle to produce energy or be converted into glucose. →Gly and three carbon to pyruvate →4 carbon to oxaloacetate →5 carbon to a-ketoglutarate

Amino Acid Transferases: Can transfer amino groups between all the amino acids (except T and K). This involves the transfer of an a-amino group from an amino acid to the a-keto position of an aketo acid. Ala ⇔ pyruvate Asp ⇔oxaloacetate Glu ⇔ a-ketoglutarate These reactions resemble redox reactions in that there must always be an amino donor and an amino acceptor. Usually these reactions are coupled to glutamate/ a-ketoglutarate. These reactions are catalyzed by aminotransferases.

Transport of nitrogen: → Apoptosis. Most NH3 is transported in blood as Gln or Ala (from the alanine cycle) to the liver. See figure 21. Nitrogens in urea come from NH3 and Asp.12 in book for the urea cycle. →Step 1: Carbamoyl phosphate is formed from ammonia. bicarbonate and ATP.and protein turnover (majority of proteins turn over every few days) lead to an excess of NH3 as proteins are degraded. The urea cycle requires 4 ATP/cycle. Free NH3 is toxic . Urea Cycle Urea is produced in a 5 step process in the liver. .programmed cell death. The urea cycle was proposed by Krebs.

AMP intermediate by the enzyme argininosuccinate synthetase. and (3) replace TCA cycle intermediates. →The main regulation is controlled by need and diet. This results in a break in the TCA cycle. →The therapy to treat any metabolic disorders that result in a decrease in the urea cycle are (1) limit protein intake. Metabolic Disorders: NH3 is toxic because excess NH3 converts a-ketoglutarate to Glu. A high protein diet and starvation activate the cycle. →Step 5: Arginine is hydrolyzed to form ornithine and urea by the enzyme arginase. → Note the "linking" of the Urea Cycle and the TCA cycle (the so-called Krebbs "bicycle"). N-acetylglutamate activates carbamoyl phosphate synthase. reduced ATP. and steps 3-5 occur in the cytosol. (This step requires 2 ATP) →Step 4: Argininosuccinate is converted to fumarate (TCA int ermediate) and arginine by the enzyme argininosuccinate lyase. →Step 3: :arginosuccinate is formed from citrulline and Aspartic acid via a citrullyl. →Steps 1 and 2 occur in the mitochondria. The ornithine can then combine with carbmoyl phosphate to begin the cycle again. (2) remove excess NH3 with a compound the will bind the NH3 and be excreted. and leads to coma and death. Tyrosine Biosynthesis . →The urea then travels to the kidneys for filtration and excretion.→Step 2: citrulline is formed from ornithine and carbamoyl phosphate by the enzyme ornithine transcarbamoylase. There is a citruline/ornithine exchange transpoter in the inner mitochondrial membrane.

50 for structures) Tyr Tyr →Melanin.autosommal recessive disorder in which phenylalanine hydroxylase is deficient.excess dopamine is responsible for psychotic symptoms and schizophrenia.skin pigment →thyroid hormone. Amino Acid Derivatives → Dopamine (neurotransmitter).neurotransmitter in brain. and this is the first step in the degradation of Phe. →Phenylketonuria. The Phe can be degraded so it is converted to phenylpyruvate (transaminase reaction) which build up in the body and causes irreversible brain damage.causes contraction of smooth muscles of arterioles and bronchioles (sleep inducing) →Melatonin –produced in the brain. This is the biosynthetic pathway for making Tyr. while lowered production of dopamine is a factor in Parkinson’s disease.sleep inducing →↑ amino acids in blood ↓ Trp to brain ↑ wakefulness →↑carbohydrates causes ↑ Trp to brain ↑ sleepiness →inhibitory neurotransmitter →converted to GABA-inhibitory neurotransmitter →histamine (decarboxylation) Trp Gly Glutamate His .Phe is converted to Tyr by phenylalanine hydroxylase.stimulates β -oxidation in cells →Seratonin. →Norpinephrine (neurotransmitter) →Epinephrine (adrenaline) (neurotransmitter) These three processes occur in the brain (see figure 26.