Ribitol-5-phosphate transferase FKTN (EC 2.7.8.-) (Fukutin) (Fukuyama-type congenital muscular dystrophy protein) (Ribitol-5-phosphate transferase)
1_MSRIN 6_ KNVVL 11_ ALLTL 16_ TSSAF 21_ LLFQL 26_ YYYKH 31_ YLSTK 36_ NGAGL 41_ SKSKG 46_ SRIGF 51_ DSTQW 56_ RAVKK 61_ FIMLT 66_ SNQNV 71_ PVFLI 76_ DPLIL 81_ ELINK 86_ NFEQV 91_ KNTSH 96_ GSTSQ 101_ CKFFC 106_ VPRDF 111_ TAFAL 116_ QYHLW 121_ KNEEG 126_ WFRIA 131_ ENMGF 136_ QCLKI 141_ ESKDP 146_ RLDGI 151_ DSLSG 156_ TEIPL 161_ HYICK 166_ LATHA 171_ IHLVV 176_ FHERS 181_ GNYLW 186_ HGHLR 191_ LKEHI 196_ DRKFV 201_ PFRKL 206_ QFGRY 211_ PGAFD 216_ RPELQ 221_ QVTVD 226_ GLEVL 231_ IPKDP 236_ MHFVE 241_ EVPHS 246_ RFIEC 251_ RYKEA 256_ RAFFQ 261_ QYLDD 266_ NTVEA 271_ VAFRK 276_ SAKEL 281_ LQLAA 286_ KTLNK 291_ LGVPF 296_ WLSSG 301_ TCLGW 306_ YRQCN 311_ IIPYS 316_ KDVDL 321_ GIFIQ 326_ DYKSD 331_ IILAF 336_ QDAGL 341_ PLKHK 346_ FGKVE 351_ DSLEL 356_ SFQGK 361_ DDVKL 366_ DVFFF 371_ YEETD 376_ HMWNG 381_ GTQAK 386_ TGKKF 391_ KYLFP 396_ KFTLC 401_ WTEFV 406_ DMKVH 411_ VPCET 416_ LEYIE 421_ ANYGK 426_ TWKIP 431_ VKTWD 436_ WKRSP 441_ PNVQP 446_ NGIWP 451_ ISEWD 456_EVIQL
1: Catalyzes the transfer of a ribitol-phosphate from CDP-ribitol to the distal N-acetylgalactosamine of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:26923585, PubMed:27194101, PubMed:29477842). This constitutes the first step in the formation of the ribitol 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed:17034757, PubMed:25279699, PubMed:26923585, PubMed:27194101, PubMed:29477842). Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity (PubMed:17034757). May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes (PubMed:25279699). Could be involved in brain development (Probable)