Sphingosine-1-phosphate metabolism in HD patients and two rodent models has shown aberrant signaling of intermediates and metabolizing enzymes, with increased expression of sphingosine-1-phosphate lyase and decreased expression of sphingosine kinase 1/2 in the striatum of post-mortem humans and HD transgenic models, both in early and late stages of the HD rodent model [94]. This evidence concerns the gene SGPL1 and Huntington disease.