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A product of a recently identified causative gene for dystroglycanopathy, AGO61, acted <jats:italic>in vitro<\/jats:italic> as a protein <jats:italic>O<\/jats:italic>-mannose \u03b2-1, 4-<jats:italic>N<\/jats:italic>-acetylglucosaminyltransferase, although it was not functionally characterized. Here we show the phenotypes of AGO61-knockout mice and demonstrate that AGO61 is indispensable for the formation of laminin-binding glycans of \u03b1-DG. AGO61-knockout mouse brain exhibited abnormal basal lamina formation and a neuronal migration defect due to a lack of laminin-binding glycans. Furthermore, our results indicate that functional \u03b1-DG glycosylation was primed by AGO61-dependent GlcNAc modifications of specific threonine-linked mannosyl moieties of \u03b1-DG. These findings provide a key missing link for understanding how the physiologically critical glycan motif is displayed on \u03b1-DG and provides new insights on the pathological mechanisms of dystroglycanopathy.<\/jats:p>","DOI":"10.1038\/srep03288","type":"journal-article","created":{"date-parts":[[2013,11,21]],"date-time":"2013-11-21T10:23:59Z","timestamp":1385029439000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["AGO61-dependent GlcNAc modification primes the formation of functional glycans on \u03b1-dystroglycan"],"prefix":"10.1038","volume":"3","author":[{"given":"Hirokazu","family":"Yagi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Nakagawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuya","family":"Saito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Kiyonari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takaya","family":"Abe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsushi","family":"Toda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sz-Wei","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay-Hooi","family":"Khoo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shogo","family":"Oka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Kato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,11,21]]},"reference":[{"key":"BFsrep03288_CR1","doi-asserted-by":"publisher","first-page":"15457","DOI":"10.1074\/jbc.R200031200","volume":"278","author":"DE Michele","year":"2003","unstructured":"Michele, D. 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