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Items: 1 to 20 of 103


Cystathionine protects against endoplasmic reticulum stress-induced lipid accumulation, tissue injury, and apoptotic cell death.

Maclean KN, Greiner LS, Evans JR, Sood SK, Lhotak S, Markham NE, Stabler SP, Allen RH, Austin RC, Balasubramaniam V, Jiang H.

J Biol Chem. 2012 Sep 14;287(38):31994-2005. doi: 10.1074/jbc.M112.355172. Epub 2012 Aug 1.


Characterization of two pathogenic mutations in cystathionine beta-synthase: different intracellular locations for wild-type and mutant proteins.

Casique L, Kabil O, Banerjee R, Martinez JC, De Lucca M.

Gene. 2013 Nov 15;531(1):117-24. doi: 10.1016/j.gene.2013.08.021. Epub 2013 Aug 24.


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 Oct-Nov;101(2-3):153-62. doi: 10.1016/j.ymgme.2010.06.010. Epub 2010 Jun 23.


Neutral aminoaciduria in cystathionine β-synthase-deficient mice; an animal model of homocystinuria.

Akahoshi N, Kamata S, Kubota M, Hishiki T, Nagahata Y, Matsuura T, Yamazaki C, Yoshida Y, Yamada H, Ishizaki Y, Suematsu M, Kasahara T, Ishii I.

Am J Physiol Renal Physiol. 2014 Jun 15;306(12):F1462-76. doi: 10.1152/ajprenal.00623.2013. Epub 2014 Apr 23.


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;103(4):330-7. doi: 10.1016/j.ymgme.2011.04.012. Epub 2011 May 5.


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;105(3):395-403. doi: 10.1016/j.ymgme.2011.11.190. Epub 2011 Dec 2.


Integrated stress response modulates cellular redox state via induction of cystathionine γ-lyase: cross-talk between integrated stress response and thiol metabolism.

Dickhout JG, Carlisle RE, Jerome DE, Mohammed-Ali Z, Jiang H, Yang G, Mani S, Garg SK, Banerjee R, Kaufman RJ, Maclean KN, Wang R, Austin RC.

J Biol Chem. 2012 Mar 2;287(10):7603-14. doi: 10.1074/jbc.M111.304576. Epub 2012 Jan 3.


Cystathionine beta synthase expression in mouse retina.

Markand S, Tawfik A, Ha Y, Gnana-Prakasam J, Sonne S, Ganapathy V, Sen N, Xian M, Smith SB.

Curr Eye Res. 2013 May;38(5):597-604. doi: 10.3109/02713683.2013.774024. Epub 2013 Mar 7.


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;107(1-2):55-65. doi: 10.1016/j.ymgme.2012.04.025. Epub 2012 May 5.


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. doi: 10.1096/fj.14-253633. Epub 2014 Jun 2.


Cystathionine beta synthase deficiency promotes oxidative stress, fibrosis, and steatosis in mice liver.

Robert K, Nehmé J, Bourdon E, Pivert G, Friguet B, Delcayre C, Delabar JM, Janel N.

Gastroenterology. 2005 May;128(5):1405-15.


Ischemia-reperfusion reduces cystathionine-beta-synthase-mediated hydrogen sulfide generation in the kidney.

Xu Z, Prathapasinghe G, Wu N, Hwang SY, Siow YL, O K.

Am J Physiol Renal Physiol. 2009 Jul;297(1):F27-35. doi: 10.1152/ajprenal.00096.2009. Epub 2009 May 13.


Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment.

Maclean KN, Sikora J, Kožich V, Jiang H, Greiner LS, Kraus E, Krijt J, Crnic LS, Allen RH, Stabler SP, Elleder M, Kraus JP.

Mol Genet Metab. 2010 Oct-Nov;101(2-3):163-71. doi: 10.1016/j.ymgme.2010.06.007. Epub 2010 Jun 22.


The presence of a transsulfuration pathway in the lens: a new oxidative stress defense system.

Persa C, Pierce A, Ma Z, Kabil O, Lou MF.

Exp Eye Res. 2004 Dec;79(6):875-86.


1,25-dihydroxyvitamin D3 influences cellular homocysteine levels in murine preosteoblastic MC3T3-E1 cells by direct regulation of cystathionine β-synthase.

Kriebitzsch C, Verlinden L, Eelen G, van Schoor NM, Swart K, Lips P, Meyer MB, Pike JW, Boonen S, Carlberg C, Vitvitsky V, Bouillon R, Banerjee R, Verstuyf A.

J Bone Miner Res. 2011 Dec;26(12):2991-3000. doi: 10.1002/jbmr.493.


Inhibition of PKR protects against tunicamycin-induced apoptosis in neuroblastoma cells.

Vaughn LS, Snee B, Patel RC.

Gene. 2014 Feb 15;536(1):90-6. doi: 10.1016/j.gene.2013.11.074. Epub 2013 Dec 14.


Increased susceptibility to acute kidney injury due to endoplasmic reticulum stress in mice lacking tumor necrosis factor-α and its receptor 1.

Huang L, Zhang R, Wu J, Chen J, Grosjean F, Satlin LH, Klein JD, Sands JM, Striker GE, Tan J, Zheng F.

Kidney Int. 2011 Mar;79(6):613-23. doi: 10.1038/ki.2010.469. Epub 2010 Dec 8.


Hyperhomocysteinemia due to cystathionine beta synthase deficiency induces dysregulation of genes involved in hepatic lipid homeostasis in mice.

Hamelet J, Demuth K, Paul JL, Delabar JM, Janel N.

J Hepatol. 2007 Jan;46(1):151-9. Epub 2006 Sep 22.


High homocysteine and thrombosis without connective tissue disorders are associated with a novel class of cystathionine beta-synthase (CBS) mutations.

Maclean KN, Gaustadnes M, Oliveriusová J, Janosík M, Kraus E, Kozich V, Kery V, Skovby F, Rüdiger N, Ingerslev J, Stabler SP, Allen RH, Kraus JP.

Hum Mutat. 2002 Jun;19(6):641-55.


Pre-steady-state kinetic analysis of enzyme-monitored turnover during cystathionine β-synthase-catalyzed H(2)S generation.

Singh S, Ballou DP, Banerjee R.

Biochemistry. 2011 Jan 25;50(3):419-25. Epub 2010 Dec 29.

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