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Varki A, Cummings RD, Esko JD, et al., editors. Essentials of Glycobiology [Internet]. 4th edition. Cold Spring Harbor (NY): Cold Spring Harbor Laboratory Press; 2022.

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Essentials of Glycobiology [Internet]. 4th edition.

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Online Appendix 27AGlycosylation Genes Essential for Mouse Embryonic Development

, PhD, , PhD, and , PhD.

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Table 1

GeneGlycan substrate accumulatedGlycan(s) not generatedStage of deathReference(s)
N-glycans
Mpi fructose-6-PMan-6-P~E11.5 DeRossi et al. 2006
Pmm2 Man-6-PMan-1-P~E3.5 Thiel et al. 2006
Nus1 farnesyl diphosphatepolyprenyl disphosphate~E7.5 Rana et al. 2016
Srd5a3 polyprenoldolicholE8.5 Cantagrel et al. 2010
Dpagt1 Dol-PGlcNAc-P-P-DolE4-5 Marek et al. 1999
Dad1 Glc3Man9GlcNAc2-PP-DolGlc3Man9GlcNAc2AsnE3.5 Brewster et al. 2000
Tmem258 Glc3Man9GlcNAc2-PP-DolGlc3Man9GlcNAc2Asn Graham et al. 2016
Uggt1 Man9GlcNAc2AsnGlc1Man9GlcNAc2AsnE13 Molinari et al. 2005
Mgat1 Man5GlcNAc2AsnGlcNAc-Man5GlcNAc2Asn~E9.5Ioffe and Stanley 1994; Metzler et al. 1994
Man2a1+
Man2a2		GlcNAcMan5GlcNAc2AsnGlcNAc-Man3GlcNAc2AsnE15.5–18.5–P0 Akama et al. 2006
Mgat2 GlcNAc-Man3GlcNAc2AsnGlcNAc2-Man3GlcNAc2AsnE9–15 Wang et al. 2001
Ngly1 N-glycosylated proteinfree oligosaccharideE16.5–P0 Fujihira et al. 2017
GPI-anchors
Piga PIGlcNAc-PIE7.5–9.5 Nozaki et al. 1999
Pgap1 inositol-acylated GPIInositol-deacylated GPIP0 Ueda et al. 2007
Pgap6 GPI-anchored proteinlyso-GPI-anchored protein~E10 Lee et al. 2016
O-GalNAc
glycans
C1galt1 GalNAc-O-Ser/ThrGal-GalNAc-Ser/Thr~E13.5 Xia et al. 2004
C1galt1c1 GalNAc-O-Ser/ThrGal-GalNAc-Ser/Thr~E13.5 Wang et al. 2010
O-fucose
glycans
Pofut1 C2XXXXS/TC3 EGF repeatsFuc-O-EGF~E9.5 Shi and Stanley 2003
Lfng Fuc-O-EGFGlcNAc-Fuc-O-EGF≤P0Evrard et al. 1998; Zhang and Gridley 1998
Pofut2 TSR type 1 repeatsFuc-O-TSR type 1~E10.5 Du et al. 2010
O-mannose
glycans
Pomt1 unmodified Ser/ThrMan-O-Ser/ThrE7.5–9.5 Willer et al. 2004
Pomt2 unmodified Ser/ThrMan-O-Ser/Thr~E9.5 Hu et al. 2011
Fktn GalNAc-GlcNAc-Man(P)-O-Ser/ThrRbo5P-GalNAc-GlcNAc-Man(P)-O-Ser/ThrE9.5 Kurahashi et al. 2005
Fkrp Rbo5P-GalNAc-GlcNAc-Man(P)-O-Ser/ThrRbo5P-Rbo5P-GalNAc-GlcNAc-Man(P)-O-Ser/Thr~E12.5 Chan et al. 2010
B4gat1 Xyl-Rbo5P-Rbo5P-GalNAc-GlcNAc-Man(P)-O-Ser/Thrpriming GlcA for XlyGlcA matriglycan added by LARGE~E9.5Wright et al. 2012; Willer et al. 2014; Kanagawa et al. 2016
O-glucose
glycans
Poglut1 C1XSXPTC2 EGF repeatsGlc-O-EGF repeat~E10.5 Fernandez-Valdivia et al. 2011
Collagen-O-galactose glycans
Plod3 Xaa-Lys-Gly on collagenhydroxylysine on collagenE9.5–E14.5 Ruotsalainen et al. 2006
Glycosaminoglycans (GAGs)
Fam20b Gal-Xyl-O-Serphospho-Gal-Xyl-O-SerE13.5 Vogel et al. 2012
B3gat3 Gal-(phospho)Gal-Xyl-O-SerGlcA-Gal-(phospho)Gal-Xyl-O-Ser~E2.5 Izumikawa et al. 2010
Extl3 GlcA-Gal-Gal-Xyl-O-SerGlcNAc-GlcA-Gal-Gal-Xyl-O-Ser~E9 Takahashi et al. 2009
Ext1 core GAG lacks HSHSE6.5–8.5 Lin et al. 2000
Ext2 core GAG lacks HSHSE6.5–8.5 Stickens et al. 2005
Ndst1+Ndst2 no N sulfation of HSHS with GlcNSEarly embryonic Holmborn et al. 2004
Hs6st1 reduced 6-O-sulfation GlcN in HSHS with GlcN6S≥E15.5 Habuchi et al. 2007
Hs6st1+Hs2st very reduced 2-O- and 6-O-sulfation GlcN in HSHS with GlcN-O-2S and GlcN-O-6S<E15,5 Conway et al. 2011
Chst11/C4st1 reduced 4-O-sulfation of GalN in CSCS with GalN-O-4S~P0 Klüppel et al. 2005
Glycolipids
Ugcg ceramide lacks GlcGlc-Cer≤E9.5 Yamashita et al. 1999
B3gnt5 GalGlc-CerGlcNAc-Gal-Glc-Cer~E3.5 Biellmann et al. 2008
B4galt5 GlcNAcGalGlc-CerGal-GlcNAc-Gal-Glc-Cer~E10.5Kumagai et al. 2009; Nishie et al. 2010
B3galnt1 Gal-Gal-Glc-CerGalNAc-Gal-Gal-Glc-CerE3.5–4.5 Vollrath et al. 2001
O-GlcNAc
Ogt unmodified Ser/ThrGlcNAc-Ser/Thr Shafi et al. 2000
Hyaluronan
Has2 UDP-GlcNAc + UDP-GlcAhyaluronanE9.5–10 Camenisch et al. 2000
Nucleotide sugars
Gne mannosamineGCs without sialic acid~E9.5 Schwarzkopf et al. 2002
Ugdh UDP-GlcUDP-GlcA~E9.5 Garcia-Garcia and Anderson 2003
Slc35c1 GDP-FucGCs with little Fuc~P0 Hellbusch et al. 2007
Slc35d1 high UDP-GlcA and UDP-GalNAc in cytoplasmCS GAGs~P0 Hiraoka et al. 2007

Modified, with permission, from Stanley P. 2016. What have we learned from glycosyltransferase knockouts in mice? J Mol Biol 428: 3166–3182. doi:10.1016/j.jmb.2016.03.025

TSR, thrombospondin repeat; EGF, epidermal growth factor; LARGE, like-acetylglucosaminyltransferase; GC, glycoconjugate; HS, heparan sulfate; CS, chondroitin sulfate; KS, keratan sulfate.

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