Spectroscopic and kinetic studies of PKU-inducing mutants of phenylalanine hydroxylase: Arg158Gln and Glu280Lys

Journal of the American Chemical Society

Volumn 125, Issue 19, 2003, Pages 5677-5686

  Kemsley, Jyllian N ^a   Wasinger, Erik C ^a   Datta, Supratim ^b   Mitić, Nataša ^c   Acharya, Tara ^c   Hedman, Britt ^d   Caradonna, John P ^b   Hodgson, Keith O ^a,d   Solomon, Edward I ^a  

^a STANFORD UNIVERSITY   (United States)

^b Boston University   (United States)

^c YALE UNIVERSITY   (United States)

^d SLAC NATIONAL ACCELERATOR LABORATORY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CATALYSIS; REACTION KINETICS; SPECTROSCOPIC ANALYSIS; X RAY SPECTROSCOPY;

CATABOLISM;

ENZYMES;

ARGININE; GLUTAMIC ACID; GLUTAMINE; LYSINE; MUTANT PROTEIN; PHENYLALANINE 4 MONOOXYGENASE; PTERIN;

ABSORPTION SPECTROSCOPY; AMINO ACID SUBSTITUTION; ARTICLE; CARBON NUCLEAR MAGNETIC RESONANCE; CIRCULAR DICHROISM; DISEASE ACTIVITY; ENZYME ANALYSIS; HYDROGEN BOND; HYDROXYLATION; IMMUNOPRECIPITATION; NONHUMAN; PHENYLKETONURIA; PROTON NUCLEAR MAGNETIC RESONANCE; REDUCTION; ROENTGEN SPECTROSCOPY; WILD TYPE;

BINDING SITES; CIRCULAR DICHROISM; DENSITOMETRY, X-RAY; FERROUS COMPOUNDS; HYDROGEN PEROXIDE; HYDROXYLATION; KINETICS; MODELS, MOLECULAR; MUTATION; PHENYLALANINE HYDROXYLASE; PHENYLKETONURIAS; RECOMBINANT PROTEINS; STRUCTURE-ACTIVITY RELATIONSHIP;

EID: 0037620614     PISSN: 00027863     EISSN: None     Source Type: Journal    
DOI: 10.1021/ja029106f     Document Type: Article

References (66)

1. Kaufman, S. Adv. Enzymol. 1993, 67, 77-264.
2. Kappock, T. J.; Caradonna, J. P. Chem. Rev. 1996, 96, 2659-2756.
3. Fitzpatrick, P. F. Annu. Rev. Biochem. 1999, 68, 355-381.
4. Fitzpatrick, P. F. Adv. Enzymol. Relat. Areas Mol. Biol. 2000, 74, 235-294.
5. Kaufman, S. Adv. Enzymol. Relat. Areas Mol. Biol. 1995, 70, 103-220.
6. Kuhn, D. M.; Lovenberg, W. In Chemistry and Biochemistry of Pterins; Blakley, R. L., Benkovic, S. J., Eds.; John Wiley & Sons: New York, 1985; Vol. 2, pp 353-382.
7. Kaufman, S. Adv. Enzymol. 1971, 35, 245-319.
8. Fisher, D. B.; Kirkwood, R.; Kaufman, S. J. Biol. Chem. 1972, 247, 5161-5167.
9. Shiman, R.; Gray, D. W. J. Biol. Chem. 1980, 255, 4793-4800.
10. Gottschall, D. W.; Dietrich, R. F.; Benkovic, S. J.; Shiman, R. J. Biol. Chem. 1982, 257, 845-849.
11. Dix, T. A.; Bollag, G. E.; Domanico, P. L.; Benkovic, S. J. Biochemistry 1985, 24, 2955-2958.
12. Siegmund, H.-U.; Kaufman, S. J. Biol. Chem. 1991, 266, 2903-2910.
13. Shiman, R. J. Biol. Chem. 1980, 255, 10 029-10 032.
14. Marota, J. J. A.; Shiman, R. Biochemistry 1984, 23, 1303-1311.
15. Wallick, D. E.; Bloom, L. M.; Gaffney, B. J.; Benkovic, S. J. Biochemistry 1984, 23, 1295-1302.
16. Shiman, R.; Jones, S. H.; Gray. D. W. J. Biol. Chem. 1990, 265, 11 633-11 642.
17. Kappock, T. J.; Harkins, P. C.; Friedenberg, S.; Caradonna, J. P. J. Biol. Chem. 1995, 270, 30 532-30 544.
18. Loeb, K. E.; Westre, T. E.; Kappock, T. J.; Mitić, N.; Glasfeld, E.; Caradonna, J. P.; Hedman, B.; Hodgson, K. O.; Solomon, E. I. J. Am. Chem. Soc. 1997, 119, 1901-1915.
19. Kemsley, J. N.; Mitid, N.; Zaleski, K. L.; Caradonna, J. P.; Solomon, E. I. J. Am. Chem. Soc. 1999, 121, 1528-1536.
20. Wasinger, E.; Mitić, N.; Hedman, B.; Caradonna, J. P.; Solomon, E. I.; Hodgson, K. O. Biochemistry 2002, 41, 6211-6217.
21. Solomon, E. I.; Brunold, T. C.; Davis, M. I.; Kemsley, J. N.; Lee, S.-K.; Lehnert, N.; Neese, F.; Skulan, A. J.; Yang, Y.-S.; Zhou. J. Chem. Rev. 2000, 100, 235-349.
22. Flatmark, T.; Stevens, R. C. Chem. Rev. 1999, 99, 2137-2160.
23. Erlandsen, H.; Bjorgo, E.; Flatmark, T.; Stevens, R. C. Biochemistry 2000, 39, 2208-2217.
24. Andersen, O. A.; Flatmark, T.; Hough, E. J. Mol. Biol. 2001, 314, 279-281.
25. Andersen, O. A.; Flatmark, T.; Hough, E. J. Mol. Biol. 2002, 320, 1095-1108.
26. Tourian, A. Biochim. Biophys. Acta 1971, 242, 345-354.
27. Xia, T.; Gray, D. W.; Shiman. R. J. Biol. Chem. 1994, 269, 24 657-24 665.
28. Solomon, E. I. Inorg. Chem. 2001, 40, 3656-3669.
29. Solomon, E. I.; Decker, A.; Lehnert, N. Proc. Nat. Acad. Sci. U.S.A. 2003 (in press).
30. http://www.mcgill.ca/pahdb.
31. Okano, Y.; Eisensmith, R. C.; Güttler, F.; Lichter-Konecki, U.; Konecki, D. S.; Trefz, F. T.; Dasovich, M.; Wang, T.; Henriksen, K.; Lou, H.; Woo, S. L. C. New Engl. J. Med. 1991, 324, 1232-1238.
32. Flatmark, T.; Knappskog, P. M.; Bjørgo, E.; Martínez, A. In Chemistry and Biology of Pteridines and Folates; Pfleiderer, W., Rokos, H., Eds.; Blackwell Science: London, 1997; pp 503-508.
33. Erlandsen, H.; Fusetti, F.; Martínez, A.; Hough, E.; Flatmark, T.; Stevens, R. C. Nat. Str. Biol. 1997, 4, 995-1000.
34. Datta, S.; Acharya, T.; Mitić, N.; Li, H.; Caradonna, J. P., manuscript in preparation.
35. Dworniczak, B.; Aulehle-Scholz, C.; Horst, J. Hum. Genet. 1989, 84, 95-96.
36. Okano, Y.; Wang, T.; Eisensmith, R. C.; Steinmann, B.; Gitzelmann, R.; Woo, S. L. C. Am. J. Hum. Genet. 1990, 46, 18-25.
37. Byck, S.; Tyfield, L.; Carter, K.; Scriver, C. R. Hum. Mutat. 1997, 9, 316-321.
38. Knappskog, P. M.; Eiken, H. G.; Martínez. A.; Olafsdottir, S.; Haavik, J.; Flatmark, T.; Apold, J. Adv. Exp. Med. Biol. 1993, 338, 59-62.
39. II}PAH unless otherwise indicated.
40. Breitmaier, E.; Gassenmann, S. Chem. Ber. 1971, 104, 665-667.
41. Stark, E.; Breitmaier, E. Tetrahedron 1973, 29, 2209-2217.
42. Moad, G.; Luthy, C. L.; Benkovic, P. A.; Benkovic, S. J. J. Am. Chem. Soc. 1979, 101, 6068-6076.
43. Maniatis, T.; Fritsch, E. F.; Sambrook, J. Molecular Cloning: A Laboratory Manual; Cold Spring Harbor Laboratory Press: Cold Spring Harbor, New York, 1982.
44. Ausubel, F. M.; Brent, R.; Kingston, R. E.; Moore. D. D.; Seidman, J. G.; Smith, J. A.; Struhl, K. Current Protocols in Molecular Biology; Wiley-Interscience: New York, 1991; Vol. 2.
45. Dix, T. A.; Benkovic, S. J. Biochemistry 1985, 24, 5839-5846.
46. Matsubara, C.; Nishikawa, Y.; Yoshida, Y.; Takamura, K. Anal. Biochem. 1983, 130, 128-133.
47. R nomenclature as for WT.
48. Browett, W. R.; Fucaloro, A. F.; Morgan, T. V.; Stephens, P. J. J. Am. Chem. Soc. 1983, 105, 1868-1872.
49. Solomon, E. I.; Pavel, E. G.; Loeb, K. E.; Campochiaro, C. Coord. Chem. Rev. 1995, 95, 369-460.
50. Pavel, E. G.; Kitajima, N.; Solomon, E. I. J. Am. Chem. Soc. 1998, 120, 3949-3962.
51. Ring conditions: 3 GeV, 50-100 mA. Beam line conditions: Rh-coated pre-monochromator, harmonic rejection and collimation mirror; 111 keV cutoff; Si(220) double-crystal monochromator fully tuned; cylindrical bent Rh-coated focusing post-monochromator mirror; energy resolution <1.4 eV.; edge position reproducibility <0.2 eV.
52. Scott, R. A.; Hahn, J. E.; Doniach, S.; Freeman, H. C.; Hodgson. K. O. J. Am. Chem. Soc. 1982, 104, 5364-5369.
53. Cramer, S. P.; Hodgson, K. O. Prog. Inorg. Chem. 1979, 25, 1-39.
54. Scott, R. A. Methods Enzymol. 1985, 117, 414-459.
55. DeWitt, J. G.; Bentsen, J. G.; Rosenzweig, A. C.; Hedman, B.; Green, J.; Pilkington, S.; Papaefthymiou, G. C.; Dalton, H.; Hodgson, K. O.; Lippard, S. J. J. Am. Chem. Soc. 1991, 113, 9219-9235.
56. Zhang, H. H.; Hedman, B.; Hodgson, K. O. In Inorganic Electronic Structure and Spectroscopy; Solomon, E. I., Lever, A. B. P., Eds.; John Wiley & Sons: New York, 1999; pp 513-554.
57. George, G. N. EDG_FIT; Stanford Synchrotron Radiation Laboratory, Stanford Linear Accelerator Center, Stanford University, Stanford, CA 94309.
58. George, G. N. EXAFSPAK; Stanford Synchrotron Radiation Laboratory, Stanford Linear Accelerator Center, Stanford University, Stanford, CA 94309.
59. Shiman, R. In Chemistry, and Biochemistry of Pterins; Blakley, R. L., Benkovic, S. J., Eds.; John Wiley & Sons: New York, 1985; Vol. 2, pp 179-249.
60. Randall, C. R.; Shu, L.; Chiou, Y.-M.; Hagen, K. S.; Ito, M.; Kitajima, N.; Lachicotte, R. J.; Zang, Y.; Que, L., Jr. Inorg. Chem. 1995, 34, 1036-1039.
61. Westre, T. E.; Kennepohl, P.; DeWitt, J. G.; Hedman, B.; Hodgson, K. O.; Solomon, E. I. J. Am. Chem. Soc. 1997, 119, 6297-6314.
62. Shu, L.; Chiou, Y.-M.; Orville, A. M.; Miller, M. A.; Lipscomb, J. D.; Que, L., Jr. Biochemistry 1995, 34, 6649-6659.
63. Davis, M. I.; Wasinger, E. C.; Westre, T. E.; Zaleski, J. M.; Orville, A. M.; Lipscomb, J. D.; Hedman, B.; Hodgson, K. 0.; Solomon, E. I. Inorg. Chem. 1999, 38, 3676-3683.
64. Francisco, W. A.; Tian, G.; Fitzpatrick, P. F.; Klinman, J. P. J. Am. Chem. Soc. 1998, 120, 4057-4062.
65. Fitzpatrick, P. F. Biochemistry 1991, 30, 3658-3662.
66. Fitzpatrick, P. F. Biochemistry 1991, 30, 6386-6391.