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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.


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.


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.


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.


Betaine supplementation is less effective than methionine restriction in correcting phenotypes of CBS deficient mice.

Gupta S, Wang L, Kruger WD.

J Inherit Metab Dis. 2016 Jan;39(1):39-46. doi: 10.1007/s10545-015-9883-z. Epub 2015 Aug 1.


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.


Homocystinuria--the effects of betaine in the treatment of patients not responsive to pyridoxine.

Wilcken DE, Wilcken B, Dudman NP, Tyrrell PA.

N Engl J Med. 1983 Aug 25;309(8):448-53.


Cystathionine beta-synthase deficiency: effects of betaine supplementation after methionine restriction in B6-nonresponsive homocystinuria.

Singh RH, Kruger WD, Wang L, Pasquali M, Elsas LJ 2nd.

Genet Med. 2004 Mar-Apr;6(2):90-5.


High homocysteine induces betaine depletion.

Imbard A, Benoist JF, Esse R, Gupta S, Lebon S, de Vriese AS, de Baulny HO, Kruger W, Schiff M, Blom HJ.

Biosci Rep. 2015 Apr 28;35(4). pii: e00222. doi: 10.1042/BSR20150094.


Effects of betaine in a murine model of mild cystathionine-beta-synthase deficiency.

Schwahn BC, Wendel U, Lussier-Cacan S, Mar MH, Zeisel SH, Leclerc D, Castro C, Garrow TA, Rozen R.

Metabolism. 2004 May;53(5):594-9.


Inhibition of betaine-homocysteine S-methyltransferase in rats causes hyperhomocysteinemia and reduces liver cystathionine β-synthase activity and methylation capacity.

Strakova J, Gupta S, Kruger WD, Dilger RN, Tryon K, Li L, Garrow TA.

Nutr Res. 2011 Jul;31(7):563-71. doi: 10.1016/j.nutres.2011.06.004.


Methionine transamination in patients with homocystinuria due to cystathionine beta-synthase deficiency.

Tangerman A, Wilcken B, Levy HL, Boers GH, Mudd SH.

Metabolism. 2000 Aug;49(8):1071-7.


Human homocysteine catabolism: three major pathways and their relevance to development of arterial occlusive disease.

Dudman NP, Guo XW, Gordon RB, Dawson PA, Wilcken DE.

J Nutr. 1996 Apr;126(4 Suppl):1295S-300S.


Dietary practices in pyridoxine non-responsive homocystinuria: a European survey.

Adam S, Almeida MF, Carbasius Weber E, Champion H, Chan H, Daly A, Dixon M, Dokoupil K, Egli D, Evans S, Eyskens F, Faria A, Ferguson C, Hallam P, Heddrich-Ellerbrok M, Jacobs J, Jankowski C, Lachmann R, Lilje R, Link R, Lowry S, Luyten K, MacDonald A, Maritz C, Martins E, Meyer U, Müller E, Murphy E, Robertson LV, Rocha JC, Saruggia I, Schick P, Stafford J, Stoelen L, Terry A, Thom R, van den Hurk T, van Rijn M, van Teefelen-Heithoff A, Webster D, White FJ, Wildgoose J, Zweers H.

Mol Genet Metab. 2013 Dec;110(4):454-9. doi: 10.1016/j.ymgme.2013.10.003. Epub 2013 Oct 10.


High casein diet decreases plasma homocysteine concentration in rats.

Ohuchi S, Matsumoto Y, Morita T, Sugiyama K.

J Nutr Sci Vitaminol (Tokyo). 2009 Feb;55(1):22-30.


Mice deficient in cystathionine beta synthase display altered homocysteine remethylation pathway.

Alberto JM, Hamelet J, Noll C, Blaise S, Bronowicki JP, Guéant JL, Delabar JM, Janel N.

Mol Genet Metab. 2007 Aug;91(4):396-8. Epub 2007 Jun 11.


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