A recessive HSP characterised by lower limbs weakness, spasticity, declined brain development, bilateral congenital cataract, jaw jerks, and tendons reflexes is observed due to mutations in the non-lysosomal glucosylceramidase β2 (SPG46/GBA2) located in the ER and Golgi apparatus membrane to cleave glucosylceramide located in neurons of cortex region at higher concentrations. This evidence concerns the gene GBA2 and hereditary spastic paraplegia.