Self Assessment: Amino Acid Synthesis Lecture 31 1. Please(name(the(essential(amino(acids:(a. Phenylalanine(b. Valine(c. Threonine(d. Tryptophan(e. Isoleucine(f. Methionine(g. Histidine(h. Arginine((i. Leucine(j. Lysine(((2. Please(name(the(3(branched(chain(amino(acids(broken(down(in(muscle(for(fuel.((a. Valine(b. Isoleucine((c. Leucine((3. What(disease(is(caused(by(a(failure(in(branched(chain(catabolism?(Maple(Syrup(Urine(Disease((4. What(are(some(symptoms(of(this(disease?((a. Marked((cognitive(deficiency(b. Failure(to(thrive(((5. What(is(the(basis(for(the(disease,(PKU? (a. Autosomal(recessive(mutation(in(the(gene(encoding(phenylalanine(hydroxylase((b. Insufficient(concentrations(of(tetrahydrobiopterin((This(results(in(lack(of,(or(reduction(in,(conversion(of(Phe(to(Tyr.(This(causes(a(build(up(of(Phe(and(conversion(to(phenylpyruvate((6. What(is(the(treatment(for(PKU?(Reduction(of(Phe(in(the(diet(and(supplement(with(Tyr((7. If(untreated,(PKU(produces(what(physiologic(problems?((a. Marked((cognitive(deficiency(b. Mousy(odor(in(urine,(sweat((c. Albinism((8. Please(state(the(basis(and(symptoms(of(Alkaptonuria:___(a. Basis:(Lack(of(homogentisate(oxigenase(b. Symptoms:(Black(urine((9. Please(list(the(amino(acids(which(are(ketogenic(only(a. Leucine______________________________________________(b. Lysine_____________________________________((10. Please(list(the(amino(acids(which(are(both(ketogenic(and(glucogenic(Self Assessment: Amino Acid Synthesis Lecture 31 a. Phenylalanine_______________________________________________(b. Tryptophan__________________________________________(c. Isoleucine_______________________________________________(d. Tyrosine_________________________________________________((11. Please(list(the(main(sources(of(nitrogen(for(biologic(systems(a. _Nitrogen(fixation(from(N2(by(bacteria(and(archea_____________________(b. __Splitting(of(N2(by(lightning___________________(c. __Industrial(sources__________________((12. Please(list((two(major(classes(of(metabolites(which(require(nitrogen(for(their(synthesis:(a)_______Amino(Acids_____________b)________Nucleotides___________((13. Please(name(the(enzyme(which(splits(and(reduces(atmospheric(oxygen(in(bacteria:___________Nitrogenase_____________________((14. Which(enzyme(is(the(prime(regulator(of(nitrogen(flow(in(the(cell((and(in(biosystems(in(general)?(_________Glutamine(Synthetase_______________((15. A(neuron_specific(isoform(of(the(enzyme(in(Q15(is(constitutively(active(–(what(is(the(benefit(of(this?______________Reduction(in(Ammonia,(Regulation(of(levels(of(Glutamate___((16. Please(state(the(function(of(SAM:__SAM(is(a(source(of(activated(methyl(groups(for(methyl(transfer_______________(17. Build_up(of(this(intermediate(of(methionine(synthesis(results(in(higher(risk(for(coronary(heart(disease___________Homocysteine___________________((18. Which(of(the(following(is(NOT(amino(acid(derivative:(a. Serotonin(b. Beta'Carotene,c. Glutathione(d. Norepinephrine(e.
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