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Journal Abstract Search

240 related items for PubMed ID: 29101223

  • 1. Taurine treatment prevents derangement of the hepatic γ-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: regulatory crosstalk between thiol and sulfinic acid metabolism.
    Maclean KN, Jiang H, Aivazidis S, Kim E, Shearn CT, Harris PS, Petersen DR, Allen RH, Stabler SP, Roede JR.
    FASEB J; 2018 Mar; 32(3):1265-1280. PubMed ID: 29101223
    [Abstract] [Full Text] [Related]

  • 2. Taurine alleviates repression of betaine-homocysteine S-methyltransferase and significantly improves the efficacy of long-term betaine treatment in a mouse model of cystathionine β-synthase-deficient homocystinuria.
    Maclean KN, Jiang H, Phinney WN, Keating AK, Hurt KJ, Stabler SP.
    FASEB J; 2019 May; 33(5):6339-6353. PubMed ID: 30768359
    [Abstract] [Full Text] [Related]

  • 3. Altered hepatic sulfur metabolism in cystathionine β-synthase-deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways.
    Jiang H, Stabler SP, Allen RH, Abman SH, Maclean KN.
    FASEB J; 2014 Sep; 28(9):4044-54. PubMed ID: 24891521
    [Abstract] [Full Text] [Related]

  • 4. Derangement of hepatic polyamine, folate, and methionine cycle metabolism in cystathionine beta-synthase-deficient homocystinuria in the presence and absence of treatment: Possible implications for pathogenesis.
    Maclean KN, Jiang H, Phinney WN, Mclagan BM, Roede JR, Stabler SP.
    Mol Genet Metab; 2021 Feb; 132(2):128-138. PubMed ID: 33483253
    [Abstract] [Full Text] [Related]

  • 5. Dysregulation of hepatic one-carbon metabolism in classical homocystinuria: Implications of redox-sensitive DHFR repression and tetrahydrofolate depletion for pathogenesis and treatment.
    Maclean KN, Jiang H, Neill PD, Chanin RR, Hurt KJ, Orlicky DJ, Bottiglieri T, Roede JR, Stabler SP.
    FASEB J; 2024 Jul 15; 38(13):e23795. PubMed ID: 38984928
    [Abstract] [Full Text] [Related]

  • 6. Enzyme replacement prevents neonatal death, liver damage, and osteoporosis in murine homocystinuria.
    Majtan T, Hůlková H, Park I, Krijt J, Kožich V, Bublil EM, Kraus JP.
    FASEB J; 2017 Dec 15; 31(12):5495-5506. PubMed ID: 28821635
    [Abstract] [Full Text] [Related]

  • 7. Cystathionine beta-synthase deficiency alters hepatic phospholipid and choline metabolism: Post-translational repression of phosphatidylethanolamine N-methyltransferase is a consequence rather than a cause of liver injury in homocystinuria.
    Jacobs RL, Jiang H, Kennelly JP, Orlicky DJ, Allen RH, Stabler SP, Maclean KN.
    Mol Genet Metab; 2017 Apr 15; 120(4):325-336. PubMed ID: 28291718
    [Abstract] [Full Text] [Related]

  • 8. Long-term betaine therapy in a murine model of cystathionine beta-synthase deficient homocystinuria: decreased efficacy over time reveals a significant threshold effect between elevated homocysteine and thrombotic risk.
    Maclean KN, Jiang H, Greiner LS, Allen RH, Stabler SP.
    Mol Genet Metab; 2012 Mar 15; 105(3):395-403. PubMed ID: 22192524
    [Abstract] [Full Text] [Related]

  • 9. Effect of acute betaine administration on hepatic metabolism of S-amino acids in rats and mice.
    Kim SK, Choi KH, Kim YC.
    Biochem Pharmacol; 2003 May 01; 65(9):1565-74. PubMed ID: 12732369
    [Abstract] [Full Text] [Related]

  • 10. Altered expression of apoA-I, apoA-IV and PON-1 activity in CBS deficient homocystinuria in the presence and absence of treatment: possible implications for cardiovascular outcomes.
    Jiang H, Stabler SP, Allen RH, Maclean KN.
    Mol Genet Metab; 2012 Sep 01; 107(1-2):55-65. PubMed ID: 22633282
    [Abstract] [Full Text] [Related]

  • 11. Enzyme replacement with PEGylated cystathionine β-synthase ameliorates homocystinuria in murine model.
    Bublil EM, Majtan T, Park I, Carrillo RS, Hůlková H, Krijt J, Kožich V, Kraus JP.
    J Clin Invest; 2016 Jun 01; 126(6):2372-84. PubMed ID: 27183385
    [Abstract] [Full Text] [Related]

  • 12. Altered gene expression in the liver of gamma-glutamyl transpeptidase-deficient mice.
    Habib GM, Shi ZZ, Ou CN, Kala G, Kala SV, Lieberman MW.
    Hepatology; 2000 Sep 01; 32(3):556-62. PubMed ID: 10960449
    [Abstract] [Full Text] [Related]

  • 13. Deciphering pathophysiological mechanisms underlying cystathionine beta-synthase-deficient homocystinuria using targeted metabolomics, liver proteomics, sphingolipidomics and analysis of mitochondrial function.
    Majtan T, Olsen T, Sokolova J, Krijt J, Křížková M, Ida T, Ditrói T, Hansikova H, Vit O, Petrak J, Kuchař L, Kruger WD, Nagy P, Akaike T, Kožich V.
    Redox Biol; 2024 Jul 01; 73():103222. PubMed ID: 38843767
    [Abstract] [Full Text] [Related]

  • 14. Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria.
    Majtan T, Jones W, Krijt J, Park I, Kruger WD, Kožich V, Bassnett S, Bublil EM, Kraus JP.
    Mol Ther; 2018 Mar 07; 26(3):834-844. PubMed ID: 29398487
    [Abstract] [Full Text] [Related]

  • 15. Classical homocystinuria: From cystathionine beta-synthase deficiency to novel enzyme therapies.
    Bublil EM, Majtan T.
    Biochimie; 2020 Jun 07; 173():48-56. PubMed ID: 31857119
    [Abstract] [Full Text] [Related]

  • 16. Constitutive induction of pro-inflammatory and chemotactic cytokines in cystathionine beta-synthase deficient homocystinuria.
    Keating AK, Freehauf C, Jiang H, Brodsky GL, Stabler SP, Allen RH, Graham DK, Thomas JA, Van Hove JL, Maclean KN.
    Mol Genet Metab; 2011 Aug 07; 103(4):330-7. PubMed ID: 21601502
    [Abstract] [Full Text] [Related]

  • 17. A novel transgenic mouse model of CBS-deficient homocystinuria does not incur hepatic steatosis or fibrosis and exhibits a hypercoagulative phenotype that is ameliorated by betaine treatment.
    Maclean KN, Sikora J, Kožich V, Jiang H, Greiner LS, Kraus E, Krijt J, Overdier KH, Collard R, Brodsky GL, Meltesen L, Crnic LS, Allen RH, Stabler SP, Elleder M, Rozen R, Patterson D, Kraus JP.
    Mol Genet Metab; 2010 Aug 07; 101(2-3):153-62. PubMed ID: 20638879
    [Abstract] [Full Text] [Related]

  • 18. Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria.
    Majtan T, Pey AL, Gimenez-Mascarell P, Martínez-Cruz LA, Szabo C, Kožich V, Kraus JP.
    Handb Exp Pharmacol; 2018 Aug 07; 245():345-383. PubMed ID: 29119254
    [Abstract] [Full Text] [Related]

  • 19. Acute glutathione depletion induces hepatic methylglyoxal accumulation by impairing its detoxification to D-lactate.
    Masterjohn C, Mah E, Park Y, Pei R, Lee J, Manautou JE, Bruno RS.
    Exp Biol Med (Maywood); 2013 Apr 07; 238(4):360-9. PubMed ID: 23760001
    [Abstract] [Full Text] [Related]

  • 20. Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine-restricted diet or enzyme replacement therapy.
    Majtan T, Park I, Cox A, Branchford BR, di Paola J, Bublil EM, Kraus JP.
    FASEB J; 2019 Nov 07; 33(11):12477-12486. PubMed ID: 31450979
    [Abstract] [Full Text] [Related]

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