MBTPS1 and Huntington disease: In conclusion, with this study we provide further evidence that modulation of S1P metabolism may represent a strong paradigm for pursuing the development of novel and more targeted therapeutic strategy by which it is possible to restore normal sphingolipid metabolism and stimulate S1P axis, which in turn may activate pro-survival pathways and reduce mHtt aggregation with a consequent preservation of neuronal homeostasis in HD brains (Figure 8).