{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T09:23:04Z","timestamp":1791278584845,"version":"4.1.0"},"reference-count":48,"publisher":"National Academy of Sciences","issue":"44","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2014,11,4]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>Proteoglycans are cellular proteins modified with long chains of repeating sugar residues connected to serine residues within the protein core by a short tetrasaccharide linker. Proteoglycans perform critical cellular functions such as formation of the extracellular matrix, binding to a diverse array of molecules, and regulation of cell motility, adhesion, and cell\u2013cell communication. We show here that family with sequence similarity 20, member B (Fam20B) is a xylose kinase that phosphorylates a xylose sugar residue within the proteoglycan tetrasaccharide linkage. Xylose phosphorylation dramatically stimulates the activity of galactosyltransferase II (GalT-II, B3GalT6), an enzyme that adds galactose to the growing linkage. Cells lacking Fam20B cannot extend the tetrasaccharide linkage and thus have immature and nonfunctional proteoglycan, a phenotype remarkably similar to Ehlers-Danlos syndrome caused by inactivating GalT-II mutations.<\/jats:p>","DOI":"10.1073\/pnas.1417993111","type":"journal-article","created":{"date-parts":[[2014,10,21]],"date-time":"2014-10-21T02:45:44Z","timestamp":1413859544000},"page":"15723-15728","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":119,"title":["Xylose phosphorylation functions as a molecular switch to regulate proteoglycan biosynthesis"],"prefix":"10.1073","volume":"111","author":[{"given":"Jianzhong","family":"Wen","sequence":"first","affiliation":[{"name":"Departments of aPharmacology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junyu","family":"Xiao","sequence":"additional","affiliation":[{"name":"Departments of aPharmacology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meghdad","family":"Rahdar","sequence":"additional","affiliation":[{"name":"Departments of aPharmacology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biswa P.","family":"Choudhury","sequence":"additional","affiliation":[{"name":"Cellular and Molecular Medicine, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jixin","family":"Cui","sequence":"additional","affiliation":[{"name":"Departments of aPharmacology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory S.","family":"Taylor","sequence":"additional","affiliation":[{"name":"Departments of aPharmacology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey D.","family":"Esko","sequence":"additional","affiliation":[{"name":"Cellular and Molecular Medicine, and"},{"name":"Glycobiology Research and Training Center, University of California, San Diego, La Jolla, CA 92093"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jack E.","family":"Dixon","sequence":"additional","affiliation":[{"name":"Departments of aPharmacology and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2014,10,20]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1075762"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/ncb0502-e127"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.280297"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)38632-5"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1239951"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20090474"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1217817"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1002246"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1177\/0300985812453177"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.113.2.193"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a004952"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrc1649"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1086\/301726"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20081"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.05.021"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2013.04.003"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2014.01.020"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2012.11.008"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1309211110"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1997.00521.x"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2009.12.067"},{"key":"e_1_3_3_22_2","first-page":"329","article-title":"Detection of proteoglycan core proteins with glycosaminoglycan lyases and antibodies","volume":"171","author":"Couchman JR","year":"2001","unstructured":"JR Couchman, P Tapanadechopone, Detection of proteoglycan core proteins with glycosaminoglycan lyases and antibodies. Methods Mol Biol 171, 329\u2013333 (2001).","journal-title":"Methods Mol Biol"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.jbchem.a122571"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.275.13.9604"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/cwp173"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.119.4.961"},{"key":"e_1_3_3_27_2","first-page":"5","article-title":"Analysis of N- and O-linked glycans from glycoproteins using MALDI-TOF mass spectrometry","volume":"534","author":"Morelle W","year":"2009","unstructured":"W Morelle, V Faid, F Chirat, JC Michalski, Analysis of N- and O-linked glycans from glycoproteins using MALDI-TOF mass spectrometry. Methods Mol Biol 534, 5\u201321 (2009).","journal-title":"Methods Mol Biol"},{"key":"e_1_3_3_28_2","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(90)93438-Q"},{"key":"e_1_3_3_29_2","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.999"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M107339200"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M709556200"},{"key":"e_1_3_3_32_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.416909"},{"key":"e_1_3_3_33_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M205078200"},{"key":"e_1_3_3_34_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M102089200"},{"key":"e_1_3_3_35_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.520536"},{"key":"e_1_3_3_36_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.87.4.1342"},{"key":"e_1_3_3_37_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-013-1351-y"},{"key":"e_1_3_3_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2013.04.016"},{"key":"e_1_3_3_39_2","doi-asserted-by":"publisher","DOI":"10.1038\/nprot.2009.29"},{"key":"e_1_3_3_40_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1025383913444"},{"key":"e_1_3_3_41_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.0000159708.71142.c8"},{"key":"e_1_3_3_42_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.322487"},{"key":"e_1_3_3_43_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1178811"},{"key":"e_1_3_3_44_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkr218"},{"key":"e_1_3_3_45_2","doi-asserted-by":"publisher","DOI":"10.2144\/04364PF01"},{"key":"e_1_3_3_46_2","doi-asserted-by":"publisher","DOI":"10.1074\/mcp.M113.030999"},{"key":"e_1_3_3_47_2","doi-asserted-by":"publisher","DOI":"10.1016\/0008-6215(84)85242-8"},{"key":"e_1_3_3_48_2","doi-asserted-by":"publisher","DOI":"10.1515\/hsz-2012-0135"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.1417993111","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T00:08:32Z","timestamp":1649808512000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.1417993111"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,10,20]]},"references-count":48,"journal-issue":{"issue":"44","published-print":{"date-parts":[[2014,11,4]]}},"alternative-id":["10.1073\/pnas.1417993111"],"URL":"https:\/\/doi.org\/10.1073\/pnas.1417993111","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.723349698.793504490","asserted-by":"object"}]},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,10,20]]},"assertion":[{"value":"2014-10-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}