CBS and fatty liver disease: Additionally, in a transgenic model (HO mice) that expresses very low levels of CBS and high plasma concentrations of Hcy and SAH, a post-translational repression of PEMT that inversely correlates with liver steatosis is present, together with upregulation and down-regulation of phospholipid species and SAM/SAH ratios similar to those found in our CBS patients [123,124].