As of 2019, despite there being more than 50 lysosomal enzymes related to the same number of LSDs, only 12 recombinant lysosomal enzymes (agalsidase alfa, agalsidase beta, alglucosidase alfa, laronidase, elosulfase alfa, galsulfase, idursulfase, imiglucerase, sebelipase alfa, taliglucerase alfa, velaglucerase alfa, and vestronidase alfa-vjbk) have been approved by the US Food and Drug Administration and are used in ERT against a few types of LSDs (Gaucher disease, Fabry disease (FD), Pompe disease, and mucopolysaccharidosis I, II, IV, VI, and VII) [13,14]. Here, IDS is linked to Fabry disease.