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Original Article

Porphobilinogen deaminase in human erythrocytes: purification of two forms with apparent molecular weights of 40 kDa and 42 kDa

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Pages 677-684 | Received 31 Dec 1988, Accepted 06 Mar 1989, Published online: 08 Jul 2009

References

  • Battersby A R, Fookes C JR, Matcham G WJ, McDonald E J. Order to assembly of the four pyrrole rings during biosynthesis of the natural porphyrins. Chem Soc Chem Coramun 1979; 12: 539–41
  • Strand L J, Meyer U A, Felsher B F, Redeker A G, Marver H S. Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyria. J Clin Invest 1972; 51: 2530–6
  • Anderson P M, Reddy R M, Anderson K E, Des-Nick R J. Characterization of the Porphobilinogen Deaminase Deficiency in Acute Intermittent Porphyria. J Clin Invest 1981; 68: 1–12
  • Desnick R J, Ostasiewicz L T, Tishler P A, Musta-Joki P. Acute intermittent porphyria: characterization of a novel mutation in the structural gene for porphobilinogen deaminase. J Clin Invest 1985; 76: 865–74
  • Mustajoki P, Desnick R J. Genetic heterogeneity in acute intermittent prophyria: characterization and frequency of porphobilinogen deaminase mutations in Finland. Br Med J 1985; 291: 505–9
  • Wilson J HP, de Rooy F WM, te Wilde K. Acute intermittent porphyria in The Netherlands. Heterogeneity of the enzyme porphobilinogen deaminase. Neth J Med 1986; 29: 393–9
  • Nunn A VW, Gardner L C, Cox T M. Molecular forms of porphobilinogen deaminase in acute intermittent porphyria. A study by western im-munoblotting. Q J Med 1987; 64: 589–99
  • Raich N, Romeo P H, Dubart A, Beaupain D, Cohen-Solal M, Goosens M. Molecular cloning and complete primary sequence of human porphobilinogen deaminase. Nucl Acid Res 1986; 14: 5955–68
  • Wang A L, Arredondo-Vega F X, Giatnpietro P F, Smith M, Anderson W F, Desnick R J. Regional gene assignment for porphobilinogen deaminase and sterease-A4 on chromosome 11. Proc Natl Acad Sci USA 1981; 78: 5734–8
  • Grandchamp B, Beaumont C, de Verneuil H, Walter O, Nordmann Y. Genetic expression of porphobilinogen deaminase and uroporphyrinogen decarboxylase during the erythroid differentiation of mouse erythroleukemic cells. Porphyrins and Porphyrias Colloque INSERM (Libbey J, Eurotext) 1986; 134: 35–44
  • Grandchamp B, de Verneuil H, Beaumont C, Chretien S, Walter O, Normann Y. Tissue-specific expression of porphobilinogen deaminase. Two isoenzymes from a single gene. Eur J Biochem 1987; 162: 105–10
  • Chretien S, Dubart A, Beaupain D, et al. Alternative transcription and splicing of the human porphobilinogen deaminase gene result either in tissue-specific or in housekeeping expression. Proc Natl Acad Sci USA 1988; 85: 6–10
  • Meisler M H, Carter M LC. Rare structural variants of human and murine uroporphyrinogen I synthase. Proc Natl Acad Sci USA 1980; 77: 2848–52
  • Anderson P M, Desnick R J. Purification and properties of uroporphyrinogen I synthase from human erythrocytes. J Biol Chem 1980; 5: 1993–9
  • Miyagi K, Kaneshima M, Kawakami J, Nakada F, Petryka Z J, Watson J. Uroporphyrinogen I synthase from human erythrocytes: separation, purification, and properties of isoenzymes. Proc Natl Acad Sci USA 1979; 76: 172–6
  • Furnagalli S A, Kotler M L, Rosetti M V, Batle A M. Human red cell porphobilinogen deaminase. A simpler method of purification and some unusual properties. Int J Biochem 1985; 17: 484–94
  • Brown R C, Elder G H, Urquhart A J. Purification of hydroxymethylbilane synthase from human erythrocytes. Biochem Soc Trans 1985; 13: 1227–8
  • de Rooij F WM, Hamer C M, Wilson J HP. Purification of porphobilinogen deaminase from human erythrocytes by fast protein liquid chromatography. Clin Chem Acta 1987; 162: 61–8
  • Smythe E, Williams D C. A simple rapid purification for hydroxymethylbilane synthase from human erythrocytes. Biochem J 1988; 251: 237–41
  • Higuchi M, Bogorad L. The purification and properties of uroporphyrinogen I synthases and uroporphyrinogen III cosynthase. Interactions between enzymes. Ann NY Acad Sci 1975; 244: 401–18
  • Williams D C. Characterization of multiple forms of hydroxymethylbilane synthase from rat spleen. Biochem J 1984; 217: 675–83
  • Jordan P M, Shemin D. Purification and properties of Uroporphyrinogen 1 synthetase from Rhodopseudomonas spheroides. J Biol Chem 1973; 248: 1019–24
  • Sassa S, Granick S, Bickers D R, Bradlow H L, Kappas A. A micro assay for uroporphyrinogen-1-synthase, one of three abnormal enzyme activities in acute intermittent porphyria, and its application to the study of the genetics of this disease. Proc Nat Acad Sci USA 1974; 71: 732–6
  • Sassa S, Zalar G L, Kappas A. Induction of uroporphyrinogen-1-synthase and expression of the gene defect of acute intermittent porphyria in mitogen-stimulated human lymphocytes. J Clin Invest 1978; 61: 499–508
  • Peterson G L. A simplification of the protein assay method of Lowry et al., which is more generally applicable. Anal Biochem 1977; 83: 346–56
  • Laemmli U K. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 1970; 227: 680–5
  • Vestcrberg O, Gramstrup-Christensen B. Sensitive silver-staining of proteins after isoelectric focusing in agarose gels. Electrophoresis 1984; 5: 282–5
  • Kornfeld R, Kornfeld S. Comparative aspects of glycoprotein structure. Ann Rev Biochem 1976; 45: 217–37
  • Beaumont C, Grandchamp B, Bogard M, de Verneuil H, Nordmann Y. Porphobilinogen deaminase is unstable in the absence of its substrate. Biochim Biophys Acta 1986; 882: 384–8

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