Lysosomal acid glucosylceramidase (Lysosomal acid GCase) (EC 3.2.1.45) (Acid beta-glucosidase) (Alglucerase) (Beta-glucocerebrosidase) (Beta-GC) (Cholesterol glucosyltransferase) (SGTase) (EC 2.4.1.-) (Cholesteryl-beta-glucosidase) (EC 3.2.1.-) (D-glucosyl-N-acylsphingosine glucohydrolase) (Imiglucerase) (Lysosomal cholesterol glycosyltransferase) (Lysosomal galactosylceramidase) (EC 3.2.1.46) (Lysosomal glycosylceramidase)
1_MEFSS 6_ PSREE 11_ CPKPL 16_ SRVSI 21_ MAGSL 26_ TGLLL 31_ LQAVS 36_ WASGA 41_ RPCIP 46_ KSFGY 51_ SSVVC 56_ VCNAT 61_ YCDSF 66_ DPPTF 71_ PALGT 76_ FSRYE 81_ STRSG 86_ RRMEL 91_ SMGPI 96_ QANHT 101_ GTGLL 106_ LTLQP 111_ EQKFQ 116_ KVKGF 121_ GGAMT 126_ DAAAL 131_ NILAL 136_ SPPAQ 141_ NLLLK 146_ SYFSE 151_ EGIGY 156_ NIIRV 161_ PMASC 166_ DFSIR 171_ TYTYA 176_ DTPDD 181_ FQLHN 186_ FSLPE 191_ EDTKL 196_ KIPLI 201_ HRALQ 206_ LAQRP 211_ VSLLA 216_ SPWTS 221_ PTWLK 226_ TNGAV 231_ NGKGS 236_ LKGQP 241_ GDIYH 246_ QTWAR 251_ YFVKF 256_ LDAYA 261_ EHKLQ 266_ FWAVT 271_ AENEP 276_ SAGLL 281_ SGYPF 286_ QCLGF 291_ TPEHQ 296_ RDFIA 301_ RDLGP 306_ TLANS 311_ THHNV 316_ RLLML 321_ DDQRL 326_ LLPHW 331_ AKVVL 336_ TDPEA 341_ AKYVH 346_ GIAVH 351_ WYLDF 356_ LAPAK 361_ ATLGE 366_ THRLF 371_ PNTML 376_ FASEA 381_ CVGSK 386_ FWEQS 391_ VRLGS 396_ WDRGM 401_ QYSHS 406_ IITNL 411_ LYHVV 416_ GWTDW 421_ NLALN 426_ PEGGP 431_ NWVRN 436_ FVDSP 441_ IIVDI 446_ TKDTF 451_ YKQPM 456_ FYHLG 461_ HFSKF 466_ IPEGS 471_ QRVGL 476_ VASQK 481_ NDLDA 486_ VALMH 491_ PDGSA 496_ VVVVL 501_ NRSSK 506_ DVPLT 511_ IKDPA 516_ VGFLE 521_ TISPG 526_ YSIHT 531_YLWRR
1: Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose (PubMed:15916907, PubMed:24211208, PubMed:32144204, PubMed:9201993). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol (PubMed:24211208, PubMed:26724485, PubMed:32144204). GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine) (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable) (PubMed:26724485). Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol (PubMed:24211208, PubMed:26724485). Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers (PubMed:32144204). Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4-enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta-D-xyloside (PubMed:33361282)