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414 related items for PubMed ID: 2208834

  • 1. Immunochemical analysis of carbamyl phosphate synthetase I and ornithine transcarbamylase deficient livers: elevated N-acetylglutamate level in a liver lacking carbamyl phosphate synthetase protein.
    Zhang W, Holzknecht RA, Butkowski RJ, Tuchman M.
    Clin Invest Med; 1990 Aug; 13(4):183-8. PubMed ID: 2208834
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  • 3. Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome.
    Brown T, Hug G, Lansky L, Bove K, Scheve A, Ryan M, Brown H, Schubert WK, Partin JC, Lloyd-Still J.
    N Engl J Med; 1976 Apr 15; 294(16):861-7. PubMed ID: 175276
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  • 4. Carbamyl phosphate synthetase and ornithine transcarbamylase activities in enzyme-deficient human liver measured by radiochromatography and correlated with outcome.
    Tuchman M, Tsai MY, Holzknecht RA, Brusilow SW.
    Pediatr Res; 1989 Jul 15; 26(1):77-82. PubMed ID: 2771513
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  • 6. Mitochondrial carbamyl phosphate and citrulline synthesis at high matrix acetylglutamate.
    Cohen NS, Cheung CW, Kyan FS, Jones EE, Raijman L.
    J Biol Chem; 1982 Jun 25; 257(12):6898-907. PubMed ID: 7085611
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  • 9. Differential effects of N-acetylglutamate on citrulline synthesis by coupled and uncoupled mitochondria.
    Cohen NS, Cheung CW.
    Arch Biochem Biophys; 1984 Oct 25; 234(1):31-44. PubMed ID: 6486824
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  • 10. Abnormalities of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of patients with Reye's syndrome.
    Sinatra F, Yoshida T, Applebaum M, Masion Hoogenraad NJ, Sunshine P.
    Pediatr Res; 1975 Nov 25; 9(11):829-33. PubMed ID: 171618
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  • 11. [Molecular basis of urea cycle disorders].
    Matsuda I, Matsuura T, Hoshide R, Uchino T, Matsubasa T.
    Nihon Rinsho; 1993 Feb 25; 51(2):520-4. PubMed ID: 8464164
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  • 12. [Neonatal hyperammonemia due to ornithine transcarbamylase deficiency (author's transl)].
    del Valle JA, Urbón A, García MJ, Cuadrado P, Ugarte M.
    An Esp Pediatr; 1982 May 25; 16(5):416-20. PubMed ID: 7114619
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  • 13. Nucleotide pool imbalances in the livers of patients with urea cycle disorders associated with increased levels of orotic aciduria.
    Vasudevan S, Qureshi IA, Lambert M, Rao PM, Rajalakshmi S, Sarma DS.
    Biochem Mol Biol Int; 1995 Mar 25; 35(3):685-90. PubMed ID: 7773204
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  • 14. Ornithine transcarbamylase (OTC) in white blood cells.
    Nagata N, Akaboshi I, Yamamoto J, Matsuda I, Ohtsuka H, Katsuki T.
    Pediatr Res; 1980 Dec 25; 14(12):1370-3. PubMed ID: 7208155
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  • 15. Focal glycogenosis of the liver in disorders of ureagenesis: its occurrence and diagnostic significance.
    Badizadegan K, Perez-Atayde AR.
    Hepatology; 1997 Aug 25; 26(2):365-73. PubMed ID: 9252147
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  • 16. [Prenatal diagnosis of carbamyl phosphate synthetase deficiency by fetal liver biopsy].
    Murotsuki J, Uehara S, Okamura K, Yajima A, Kikuchi M, Oura T, Miyabayashi S.
    Nihon Sanka Fujinka Gakkai Zasshi; 1991 Dec 25; 43(12):1613-6. PubMed ID: 1744457
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  • 17. Orthotopic liver transplantation for urea cycle enzyme deficiency.
    Todo S, Starzl TE, Tzakis A, Benkov KJ, Kalousek F, Saheki T, Tanikawa K, Fenton WA.
    Hepatology; 1992 Mar 25; 15(3):419-22. PubMed ID: 1544622
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  • 18. N-acetylglutamate-independent activity of carbamyl phosphate synthetase (ammonia): implications for the kinetic assay of acetylglutamate.
    Cohen NS.
    Arch Biochem Biophys; 1984 Jul 25; 232(1):38-46. PubMed ID: 6742858
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  • 19. Carbamyl phosphate synthetase I deficiency. One base substitution in an exon of the CPS I gene causes a 9-basepair deletion due to aberrant splicing.
    Hoshide R, Matsuura T, Haraguchi Y, Endo F, Yoshinaga M, Matsuda I.
    J Clin Invest; 1993 May 25; 91(5):1884-7. PubMed ID: 8486760
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  • 20. Heterogeneity of patients with late onset ornithine transcarbamylase deficiency.
    Tuchman M, Holzknecht RA.
    Clin Invest Med; 1991 Aug 25; 14(4):320-4. PubMed ID: 1782729
    [Abstract] [Full Text] [Related]

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