B12 METABOLISM IN HUMANS By NICOLE AURORA LEAL A ...

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128 Del Corral, A. and R. Carmel. 1990. Transfer of cobalamin from the cobalamin-binding protein of egg yolk to R binder of human saliva and gastric juice. Gastroenterologist 98:1460-6. Dixon, M. M., S. Huang, R. G. Matthews and M. Ludwig. 1996. The structure of the C-terminal domain of methionine synthase: presenting S-adenosylmethionine for reductive methylation of B12. Structure 4:1263-75. Dobson, C. M., T. Wai, D. Leclerc, H. Kadir, M. Narang, J. P. Lerner-Ellis, T. J. Hudson, D. S. Rosenblatt and R. A. Gravel. 2002. Identification of the gene responsible for the cblB complementation group of vitamin B12-dependent methylmalonic aciduria. Hum. Mol. Genet. 11:3361-9. Dobson, C. M., T. Wai, D. Leclerc, A. Wilson, X. Wu, C. Dore, T. Hudson, D. S. Rosenblatt and R. A. Gravel. 2002. Identification of the gene responsible for the cblA complementation group of vitamin B12-responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements. Proc. Natl. Acad. Sci. U S A 99:15554-9. Dolphin, D. 1982. B12. John Wiley & Sons, Inc., New York. Drennan, C. L., S. Huang, J. T. Drummond, R. G. Matthews and M. L. Ludwig. 1994. How a protein binds B12: a 3.0 Å x-ray structure of B12-binding domains of methionine synthase. Science 266:1669-1674. Drummond, J. T. and R. G. Matthews. 1993. Cobalamin-dependent and cobalaminindependent methionine synthases in Escherichia coli: two solutions to the same chemical problem. Adv. Exp. Med. Biol. 338:687-692. Drummond, J. T. and R. G. Matthews. 1994. Nitrous oxide inactivation of cobalamindependent methionine synthase from Escherichia coli: characterization of the damage to the enzyme and prosthetic group. Biochemistry 33:3742-50. Fenton, W. A., R. A. Gravel and D. Rosenblatt. 2000. Disorders of propionate and methylmalonate metabolism. In C. R. Scriver, A. L. Beaudet, W. S. Sly, et al. (ed.), The Metabolic and molecular basis of inherited disease. McGraw-Hill, New York. Fenton, W. A. and L. E. Rosenberg. 1978a. Genetic and biochemical analysis of human cobalamin mutants in cell culture. Annu. Rev. Genet. 12:223-48. Fenton, W. A. and L. E. Rosenberg. 1978b. Mitochondrial metabolism of hydroxocobalamin: synthesis of adenosylcobalamin by intact rat liver mitochondria. Arch. Biochem. Biophys. 189:441-7. Fenton, W. A. and L. E. Rosenberg. 1981. The defect in the cblB class of human methylmalonic acidemia: deficiency of cob(I)alamin adenosyltransferase activity in extracts of cultured fibroblasts. Biochem. Biophys. Res. Commun. 98:283-289.
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B12 METABOLISM IN HUMANS By NICOLE
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The work in this dissertation is de
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TABLE OF CONTENTS Page ACKNOWLEDGME
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General Molecular and Protein Metho
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LIST OF TABLES Table page 1-1. Aden
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3-7. Stoichiometry of the MSR-ATR s
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DTT dithiothreitol E. coli Escheric
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Abstract of Dissertation Presented
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CHAPTER 1 INTRODUCTION History of C
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3 (DMB). The DMB moiety is covalent
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5 and deoxyadenosine. After hydroge
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7 homocysteine recycling and methio
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methylmalonic acid. Methylmalonic a
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folate-dependent reactions includin
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tetanomorphum, P. shermanii, Euglen
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enzyme. This suggests that cob(II)a
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at the 5’-C of ATP by cob(I)alami
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iochemical studies and complementat
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1997). Both cases of the cblD disor
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23 The cblB complementation group i
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(Watkins et al. 2002). Some patient
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Chapter 2 of this dissertation repo
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Corrin Ring Dimethyl benzimidazole
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Classes Eliminases Dehydratases OH
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A) B) CH 3THF 33 MS-cob(I)alamin Me
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propanol dehydrogenase (PduQ) propa
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or from mutations that impair the a
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1989). 5-bromo-4-chloro-3-indolyl-
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41 5’-GCCGCCGGTACCGATGACGACGACAAG
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43 Growth of Adenosyltransferase Ex
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anti-rabbit IgG (γ-chain specific)
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sequence similarity to a known ATR
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ATRs were produced as fusion protei
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51 of the lacI repressor (not shown
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in which proteins bind B12, the "ba
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libraries may be particularly usefu
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57 Figure 2-1. Multiple sequence al
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Table 2-2. Specific activities of b
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61 1 2 3 4 5 25 kDa Figure 2-4. Wes
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63 (cyanocobalamin, CNCbl) and hydr
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65 5’- triphosphate (ATP), and di
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extracts of ATR 239K (160 mg protei
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Milford, MA). Samples (200 µl) wer
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Linearity of the ATR reaction 71 Th
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73 reactions was characteristic of
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75 MSR Produces little Cob(I)alamin
Page 93 and 94: Johnson et al. 2001, Saridakis et a
Page 95 and 96: AdoCbl by the MSR-ATR system. Exper
Page 97 and 98: propionyl-CoA PCC (2S)-methylmalony
Page 99 and 100: kDa 97 66 45 31 21 14 7 83 1 2 3 4
Page 101 and 102: 85 Table 3-3. The MSR-ATR system se
Page 103 and 104: Absorbance 0.5 0.4 0.3 0.2 0.1 0.0
Page 105 and 106: Wavelength, (525 nm) 0.24 0.23 0.22
Page 107 and 108: Activity, (nmol min-1 Activity, (nm
Page 109 and 110: al. 1988). The aldehyde is then dis
Page 111 and 112: CoA-acylating propionaldehyde dehyd
Page 113 and 114: 97 previously published DNA sequenc
Page 115 and 116: mM CHES (2-[N-cyclohexylamino]ethan
Page 117 and 118: 101 For the conjugation step, strai
Page 119 and 120: 103 from New England Biolabs (Bever
Page 121 and 122: 105 Propionaldehyde Dehydrogenase A
Page 123 and 124: 107 fraction. Following centrifugat
Page 125 and 126: 109 antiserum obtained was specific
Page 127 and 128: 111 biochemical evidence was presen
Page 129 and 130: 113 Table 4-1. Bacterial strains Sp
Page 131 and 132: 115 Table 4-3. Propionaldehyde dehy
Page 133 and 134: kDa 209 80 49 35 29 117 1 2 3 4 Fig
Page 135 and 136: CHAPTER 5 CONCLUSIONS In humans, co
Page 137 and 138: 121 substitutions that are common p
Page 139 and 140: 123 Studies also indicated that hum
Page 141 and 142: LIST OF REFERENCES Aberg, A., S. Ha
Page 143: 127 Bradford, M. 1976. A rapid and
Page 147 and 148: 131 Johnson, C. L. V. J., E. Pechon
Page 149 and 150: 133 Mellman, I., H. F. Willard and
Page 151 and 152: 135 Rossi, M., J. P. Glusker, L. Ra
Page 153 and 154: 137 Vlasie, M., S. Chowdhury and R.
Page 155 and 156: BIOGRAPHICAL SKETCH Nicole Aurora L
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