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In the endoplasmic reticulum, B3GLCT adds glucose to O-linked fucose on properly folded thrombospondin type 1 repeats (TSRs). The resulting glucose\u2013fucose disaccharide is proposed to stabilize the TSR fold and promote secretion of B3GLCT substrates, with some substrates more sensitive than others to loss of glucose. Mouse B3glct mutants develop hydrocephalus at high frequency. In this study, we demonstrated that B3glct mutant ependymal cells had fewer cilia basal bodies and altered translational polarity compared to controls. Localization of mRNA encoding A Disintegrin and Metalloproteinase with ThromboSpondin type 1 repeat 20 (ADAMTS20) and ADAMTS9 suggested that reduced function of these B3GLCT substrates contributed to ependymal cell abnormalities. In addition, we showed that multiple B3GLCT substrates (Adamts3, Adamts9 and Adamts20) are expressed by the subcommissural organ, that subcommissural organ-spondin ((SSPO) also known as SCO-spondin) TSRs were modified with O-linked glucose\u2013fucose and that loss of B3GLCT reduced secretion of SSPO in cultured cells. In the B3glct mutant, intracellular levels of SSPO were reduced and BiP levels increased, suggesting a folding defect. Secreted SSPO colocalized with BiP, raising the possibility that abnormal extracellular assembly of SSPO into Reissner\u2019s fiber also contributed to impaired CSF flow in mutants. Combined, these studies underscore the complexity of the B3glct mutant hydrocephalus phenotype and demonstrate that impaired cerebrospinal fluid (CSF) flow likely stems from the collective effects of the mutation on multiple processes.<\/jats:p>","DOI":"10.1093\/glycob\/cwab033","type":"journal-article","created":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T17:34:19Z","timestamp":1618594459000},"page":"988-1004","source":"Crossref","is-referenced-by-count":15,"title":["Hydrocephalus in mouse\n                    <i>B3glct<\/i>\n                    mutants is likely caused by defects in multiple B3GLCT substrates in ependymal cells and subcommissural organ"],"prefix":"10.1093","volume":"31","author":[{"given":"Sanjiv","family":"Neupane","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Cell Biology, Stony Brook University, 450 Life Science Building, Stony Brook, NY 11794-5215, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"June","family":"Goto","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Cincinnati Children\u2019s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J","family":"Berardinelli","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Complex Carbohydrate Research Center, 315 Riverbend Road, Athens, GA 30602, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsuko","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Complex Carbohydrate Research Center, 315 Riverbend Road, Athens, GA 30602, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert S","family":"Haltiwanger","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Complex Carbohydrate Research Center, 315 Riverbend Road, Athens, GA 30602, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernadette C","family":"Holdener","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Cell Biology, Stony Brook University, 450 Life Science Building, Stony Brook, NY 11794-5215, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,4,28]]},"reference":[{"key":"2021111010000616800_ref1","doi-asserted-by":"crossref","DOI":"10.1242\/dev.154500","article-title":"A mutation in Ccdc39 causes neonatal hydrocephalus with abnormal motile cilia development in mice","volume":"145","author":"Abdelhamed","year":"2018","journal-title":"Development"},{"key":"2021111010000616800_ref2","doi-asserted-by":"crossref","first-page":"11","DOI":"10.3389\/fmolb.2019.00011","article-title":"Structure and molecular mechanism of ER stress signaling by the unfolded protein 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