Overview lysosome Function digest endosomal material via digestive enzymes login to view 3 more bullets all are acid hydrolases which function at low pH found in all cells higher concentrations in phagocytic cells Forms primary lysosomes newly formed from the trans-Golgi waiting to receive endocytosed material secondary lysosomes aka phagolysosome formed when primary lysosomes fuse with endocytic vesicles Lysosomal Storage Disease (by Deficient Enzymes) Sphingolipidoses Sphingomyelinase deficient in Niemann-Pick disease login to view 12 more bullets α-galactosidase A deficient in Fabry disease login to view 11 more bullets β-galactocerebrosidase deficient in Krabbe disease login to view 9 more bullets β-glucocerebrosidase deficient in Gaucher disease login to view 21 more bullets Hexosaminidase A deficient in Tay-Sachs disease login to view 12 more bullets Arylsulfatase A deficient in metachromatic leukodystrophy login to view 6 more bullets Mucopolysaccharides α-L-iduronidase deficient in Hurler syndrome login to view 7 more bullets Iduronate sulfatase deficient in Hunter syndrome login to view 8 more bullets Other Lysosomal Disorders I (inclusion)-cell disease cause proteins marked for localization to lysosomes are post-translationally modified in the Golgi login to view 1 more bullet defect in N-acetylglucosamine-phosphotransferase causes I-cell disease login to view 1 more bullet cells cannot degrade endocytosed material and inclusion bodies build up intracellularly presentation high plasma levels of lysosomal enzymes skeletal abnormalities restricted joint movement psychomotor retardation early death coarse facial features no treatment Chédiak–Higashi syndrome cause primary lysosomes of leukocytes cannot fuse with phagosomes due to inability of microtubles to polymerize also affects immune cell chemotaxis presentation ↑ infections login to view 1 more bullet partial albinism peripheral neuropathy inheritance AR