Gaucher disease is an autosomal recessive lysosomal storage disease caused by a deficiency of glucosylceramidase (β-glucosidase), which results in the abnormal accumulation of glucosylceramide (GlcCer) in tissues. The accumulation of glucosylsphingosine (GlcSph), a derivative of GlcCer devoid of fatty acids, has been demonstrated in the affected tissues of patients with Gaucher disease.