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 b c d Phenylalanine Tryptophan Isoleucine 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 Sphingosine
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