Interestingly, recent studies have also demonstrated that inhibiting biosynthesis of LCCs in the mouse brain due to mutation [59] or deficiency [48] in CerS1 leads to progressive cerebellar ataxia due to the degeneration of PCs; thereby suggesting that LCCs are essential for the survival of PCs, although their aberrant increase is also detrimental to these cells. Here, CERS1 is linked to aceruloplasminemia.