Huizing et al. Page 18 [91]. Charles P, Hernandez MP, Stankoff B, Aigrot MS, Colin C, Rougon G, Zalc B, Lubetzki C, A Negative regulation of central nervous system myelination by polysialylated-neural cell adhesion uthor Man molecule, Proc Natl Acad Sci U S A 97 (2000) 7585–7590. [PubMed: 10840047] [92]. Fewou SN, Ramakrishnan H, Bussow H, Gieselmann V, Eckhardt M, Down-regulation of polysialic acid is required for efficient myelin formation, J Biol Chem 282 (2007) 16700–16711. [PubMed: 17420257] uscr [93]. Aula N, Kopra O, Jalanko A, Peltonen L, Sialin expression in the CNS implicates extralysosomal function in neurons, Neurobiol Dis 15 (2004) 251–261. [PubMed: 15006695] ipt [94]. Miyaji T, Omote H, Moriyama Y, Functional characterization of vesicular excitatory amino acid transport by human sialin, J Neurochem 119 (2011) 1–5. [PubMed: 21781115] [95]. Morland C, Nordengen K, Larsson M, Prolo LM, Farzampour Z, Reimer RJ, Gundersen V, Vesicular uptake and exocytosis of L-aspartate is independent of sialin, FASEB J 27 (2013) 1264–1274. [PubMed: 23221336] [96]. Mahal LK, Yarema KJ, Bertozzi CR, Engineering chemical reactivity on cell surfaces through oligosaccharide biosynthesis, Science 276 (1997) 1125–1128. [PubMed: 9173543] A [97]. Gilormini PA, Lion C, Vicogne D, Guerardel Y, Foulquier F, Biot C, Chemical glycomics uthor Man enrichment: imaging the recycling of sialic acid in living cells, J Inherit Metab Dis 41 (2018) 515–523. [PubMed: 29294191] [98]. Gilormini PA, Lion C, Vicogne D, Levade T, Potelle S, Mariller C, Guerardel Y, Biot C, Foulquier F, A sequential bioorthogonal dual strategy: ManNAl and SiaNAl as distinct tools to unravel sialic acid metabolic pathways, Chem Commun (Camb) 52 (2016) 2318–2321. [PubMed: uscr 26727964] [99]. Marton RM, Pasca SP, Organoid and Assembloid Technologies for Investigating Cellular ipt Crosstalk in Human Brain Development and Disease, Trends Cell Biol 30 (2020) 133–143. [PubMed: 31879153] [100]. Luciani M, Gritti A, Meneghini V, Human iPSC-Based Models for the Development of Therapeutics Targeting Neurodegenerative Lysosomal Storage Diseases, Front Mol Biosci 7 (2020) 224. [PubMed: 33062642] [101]. Kido J, Nakamura K, Era T, Role of induced pluripotent stem cells in lysosomal storage diseases, Mol Cell Neurosci 108 (2020) 103540. [PubMed: 32828964] A [102]. Latour YL, Yoon R, Thomas SE, Grant C, Li C, Sena-Esteves M, Allende ML, Proia RL, Tifft uthor Man CJ, Human GLB1 knockout cerebral organoids: A model system for testing AAV9-mediated GLB1 gene therapy for reducing GM1 ganglioside storage in GM1 gangliosidosis, Mol Genet Metab Rep 21 (2019) 100513. [PubMed: 31534909] [103]. Stroobants S, Van Acker NG, Verheijen FW, Goris I, Daneels GF, Schot R, Verbeek E, Knaapen uscr MW, De Bondt A, Gohlmann HW, Crauwels ML, Mancini GM, Andries LJ, Moechars DW, D’Hooge R, Progressive leukoencephalopathy impairs neurobehavioral development in sialin- ipt deficient mice, Exp Neurol 291 (2017) 106–119. [PubMed: 28189729] [104]. Myall NJ, Wreden CC, Wlizla M, Reimer RJ, G328E and G409E sialin missense mutations similarly impair transport activity, but differentially affect trafficking, Mol Genet Metab 92 (2007) 371–374. [PubMed: 17933575] [105]. Dubois L, Pietrancosta N, Cabaye A, Fanget I, Debacker C, Gilormini PA, Dansette PM, Dairou J, Biot C, Froissart R, Goupil-Lamy A, Bertrand HO, Acher FC, McCort-Tranchepain I, Gasnier B, Anne C, Amino Acids Bearing Aromatic or Heteroaromatic Substituents as a New Class of Ligands for the Lysosomal Sialic Acid Transporter Sialin, J Med Chem 63 (2020) 8231–8249. A uthor Man [PubMed: 32608236] [106]. Teti A, Econs MJ, Osteopetroses, emphasizing potential approaches to treatment, Bone 102 (2017) 50–59. [PubMed: 28167345] [107]. Rocca CJ, Cherqui S, Potential use of stem cells as a therapy for cystinosis, Pediatr Nephrol 34 (2019) 965–973. [PubMed: 29789935] uscr [108]. Nair S, Strohecker AM, Persaud AK, Bissa B, Muruganandan S, McElroy C, Pathak R, Williams M, Raj R, Kaddoumi A, Sparreboom A, Beedle AM, Govindarajan R, Adult stem cell ipt deficits drive Slc29a3 disorders in mice, Nat Commun 10 (2019) 2943. [PubMed: 31270333] Neurosci Lett. Author manuscript; available in PMC 2021 June 11.