Also, a marked increase in the expression of CCL2 has been documented in bone marrow mesenchymal stromal cells from an adult patient with Gaucher disease type 1 [157] and in cerebrospinal fluids of patients with the severe infantile phenotype of gangliosidoses (Tay-Sachs disease, Sandhoff disease, and GM1-gangliosidosis) [113] as well as in the brain of the Gbaflox/flox; nestin-Cre [121] and Hexb−/− mouse models [158]. Here, NES is linked to Sandhoff disease.