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

Cystathionine beta-synthase (CBS) catalyzes the condensation of homocysteine (Hcy) and serine to cystathionine, which is then hydrolyzed to cysteine by cystathionine gamma-lyase. Inactivation of CBS results in CBS-deficient homocystinuria more commonly referred to as classical homocystinuria, which,...

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Detalles Bibliográficos
Autores principales: Maclean, Kenneth N., Sikora, Jakub, Kožich, Viktor, Jiang, Hua, Greiner, Lori S., Kraus, Eva, Krijt, Jakub, Overdier, Katherine H., Collard, Renata, Brodsky, Gary L., Meltesen, Lynne, Crnic, Linda S., Allen, Robert H., Stabler, Sally P., Elleder, Milan, Rozen, Rima, Patterson, David, Kraus, Jan P.
Formato: Texto
Lenguaje:English
Publicado: Academic Press 2010
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2954364/
https://www.ncbi.nlm.nih.gov/pubmed/20638879
http://dx.doi.org/10.1016/j.ymgme.2010.06.010

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2954364/
https://www.ncbi.nlm.nih.gov/pubmed/20638879
http://dx.doi.org/10.1016/j.ymgme.2010.06.010

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