Binding Site Information of Target
| Target General Information | Top | ||||
|---|---|---|---|---|---|
| Target ID | T31514 | Target Info | |||
| Target Name | Lysosomal alpha-glucosidase (GAA) | ||||
| Synonyms | GAA; Aglucosidase alfa; Acid maltase | ||||
| Target Type | Successful Target | ||||
| Gene Name | GAA | ||||
| Biochemical Class | Glycosylase | ||||
| UniProt ID | |||||
| Drug Binding Sites of Target | Top | |||||
|---|---|---|---|---|---|---|
| Ligand Name: Acetylcysteine | Ligand Info | |||||
| Structure Description | Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-acetyl-cysteine | PDB:5NN4 | ||||
| Method | X-ray diffraction | Resolution | 1.83 Å | Mutation | No | [1] |
| PDB Sequence |
QCDVPPNSRF
90 DCAPDKAITQ100 EQCEARGCCY110 IPAKQGQPWC127 FFPPSYPSYK137 LENLSSSEMG 147 YTATLTRTTP157 TFFPKDILTL167 RLDVMMETEN177 RLHFTIKDPA187 NRRYEVPLET 197 APSPLYSVEF213 SEEPFGVIVH223 RQLDGRVLLN233 TTVAPLFFAD243 QFLQLSTSLP 253 SQYITGLAEH263 LSPLMLSTSW273 TRITLWNRDL283 APTPGANLYG293 SHPFYLALED 303 GGSAHGVFLL313 NSNAMDVVLQ323 PSPALSWRST333 GGILDVYIFL343 GPEPKSVVQQ 353 YLDVVGYPFM363 PPYWGLGFHL373 CRWGYSSTAI383 TRQVVENMTR393 AHFPLDVQWN 403 DLDYMDSRRD413 FTFNKDGFRD423 FPAMVQELHQ433 GGRRYMMIVD443 PAISSSGPAG 453 SYRPYDEGLR463 RGVFITNETG473 QPLIGKVWPG483 STAFPDFTNP493 TALAWWEDMV 503 AEFHDQVPFD513 GMWIDMNEPS523 NFIRGSEDGC533 PNNELENPPY543 VPGVVGGTLQ 553 AATICASSHQ563 FLSTHYNLHN573 LYGLTEAIAS583 HRALVKARGT593 RPFVISRSTF 603 AGHGRYAGHW613 TGDVWSSWEQ623 LASSVPEILQ633 FNLLGVPLVG643 ADVCGFLGNT 653 SEELCVRWTQ663 LGAFYPFMRN673 HNSLLSLPQE683 PYSFSEPAQQ693 AMRKALTLRY 703 ALLPHLYTLF713 HQAHVAGETV723 ARPLFLEFPK733 DSSTWTVDHQ743 LLWGEALLIT 753 PVLQAGKAEV763 TGYFPLGTWY773 DLQTVPIERE795 PAIHSEGQWV805 TLPAPLDTIN 815 VHLRAGYIIP825 LQGPGLTTTE835 SRQQPMALAV845 ALTKGGEARG855 ELFWDDGESL 865 EVLERGAYTQ875 VIFLARNNTI885 VNELVRVTSE895 GAGLQLQKVT905 VLGVATAPQQ 915 VLSNGVPVSN925 FTYSPDTKVL935 DICVSLLMGE945 QFLVSWC
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| Ligand Name: Miglitol | Ligand Info | |||||
| Structure Description | Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin | PDB:5NN6 | ||||
| Method | X-ray diffraction | Resolution | 2.00 Å | Mutation | No | [1] |
| PDB Sequence |
QCDVPPNSRF
90 DCAPDKAITQ100 EQCEARGCCY110 IPAKQGQPWC127 FFPPSYPSYK137 LENLSSSEMG 147 YTATLTRTTP157 TFFPKDILTL167 RLDVMMETEN177 RLHFTIKDPA187 NRRYEVPLAP 205 SPLYSVEFSE215 EPFGVIVHRQ225 LDGRVLLNTT235 VAPLFFADQF245 LQLSTSLPSQ 255 YITGLAEHLS265 PLMLSTSWTR275 ITLWNRDLAP285 TPGANLYGSH295 PFYLALEDGG 305 SAHGVFLLNS315 NAMDVVLQPS325 PALSWRSTGG335 ILDVYIFLGP345 EPKSVVQQYL 355 DVVGYPFMPP365 YWGLGFHLCR375 WGYSSTAITR385 QVVENMTRAH395 FPLDVQWNDL 405 DYMDSRRDFT415 FNKDGFRDFP425 AMVQELHQGG435 RRYMMIVDPA445 ISSSGPAGSY 455 RPYDEGLRRG465 VFITNETGQP475 LIGKVWPGST485 AFPDFTNPTA495 LAWWEDMVAE 505 FHDQVPFDGM515 WIDMNEPSNF525 IRGSEDGCPN535 NELENPPYVP545 GVVGGTLQAA 555 TICASSHQFL565 STHYNLHNLY575 GLTEAIASHR585 ALVKARGTRP595 FVISRSTFAG 605 HGRYAGHWTG615 DVWSSWEQLA625 SSVPEILQFN635 LLGVPLVGAD645 VCGFLGNTSE 655 ELCVRWTQLG665 AFYPFMRNHN675 SLLSLPQEPY685 SFSEPAQQAM695 RKALTLRYAL 705 LPHLYTLFHQ715 AHVAGETVAR725 PLFLEFPKDS735 STWTVDHQLL745 WGEALLITPV 755 LQAGKAEVTG765 YFPLGTWYDL775 QTVPIEREPA797 IHSEGQWVTL807 PAPLDTINVH 817 LRAGYIIPLQ827 GPGLTTTESR837 QQPMALAVAL847 TKGGEARGEL857 FWDDGESLEV 867 LERGAYTQVI877 FLARNNTIVN887 ELVRVTSEGA897 GLQLQKVTVL907 GVATAPQQVL 917 SNGVPVSNFT927 YSPDTKVLDI937 VSLLMGEQFL948 VSWC
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| Ligand Name: Deoxynojirimycin | Ligand Info | |||||
| Structure Description | Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with 1-deoxynojirimycin | PDB:5NN5 | ||||
| Method | X-ray diffraction | Resolution | 2.00 Å | Mutation | No | [1] |
| PDB Sequence |
QCDVPPNSRF
90 DCAPDKAITQ100 EQCEARGCCY110 IPAKQGQPWC127 FFPPSYPSYK137 LENLSSSEMG 147 YTATLTRTTP157 TFFPKDILTL167 RLDVMMETEN177 RLHFTIKDPA187 NRRYEVPLET 197 APSPLYSVEF213 SEEPFGVIVH223 RQLDGRVLLN233 TTVAPLFFAD243 QFLQLSTSLP 253 SQYITGLAEH263 LSPLMLSTSW273 TRITLWNRDL283 APTPGANLYG293 SHPFYLALED 303 GGSAHGVFLL313 NSNAMDVVLQ323 PSPALSWRST333 GGILDVYIFL343 GPEPKSVVQQ 353 YLDVVGYPFM363 PPYWGLGFHL373 CRWGYSSTAI383 TRQVVENMTR393 AHFPLDVQWN 403 DLDYMDSRRD413 FTFNKDGFRD423 FPAMVQELHQ433 GGRRYMMIVD443 PAISSSGPAG 453 SYRPYDEGLR463 RGVFITNETG473 QPLIGKVWPG483 STAFPDFTNP493 TALAWWEDMV 503 AEFHDQVPFD513 GMWIDMNEPS523 NFIRGSEDGC533 PNNELENPPY543 VPGVVGGTLQ 553 AATICASSHQ563 FLSTHYNLHN573 LYGLTEAIAS583 HRALVKARGT593 RPFVISRSTF 603 AGHGRYAGHW613 TGDVWSSWEQ623 LASSVPEILQ633 FNLLGVPLVG643 ADVCGFLGNT 653 SEELCVRWTQ663 LGAFYPFMRN673 HNSLLSLPQE683 PYSFSEPAQQ693 AMRKALTLRY 703 ALLPHLYTLF713 HQAHVAGETV723 ARPLFLEFPK733 DSSTWTVDHQ743 LLWGEALLIT 753 PVLQAGKAEV763 TGYFPLGTWY773 DLQTVPIEEP796 AIHSEGQWVT806 LPAPLDTINV 816 HLRAGYIIPL826 QGPGLTTTES836 RQQPMALAVA846 LTKGGEARGE856 LFWDDGESLE 866 VLERGAYTQV876 IFLARNNTIV886 NELVRVTSEG896 AGLQLQKVTV906 LGVATAPQQV 916 LSNGVPVSNF926 TYSPDTKVLD936 IVSLLMGEQF947 LVSWC
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Click to Show 3D Structure of This Binding Site
set background white;style ions nothing; color 8e8e8e;style chemicals nothing; select .NOJ or .NOJ2 or .NOJ3 or :3NOJ;style chemicals stick;color identity;select .A:376 or .A:404 or .A:405 or .A:441 or .A:481 or .A:516 or .A:518 or .A:519 or .A:600 or .A:613 or .A:615 or .A:616 or .A:645 or .A:649 or .A:672 or .A:674; color #00ffc7; zoom selection;set surface opacity 0.5;set surface Van der Waals surface;set mode all
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| Ligand Name: Beta-D-Glucose | Ligand Info | |||||
| Structure Description | Crystal structure of human GAA | PDB:5KZW | ||||
| Method | X-ray diffraction | Resolution | 2.00 Å | Mutation | No | [2] |
| PDB Sequence |
PTQCDVPPNS
88 RFDCAPDKAI98 TQEQCEARGC108 CYIPAKQMGQ124 PWCFFPPSYP134 SYKLENLSSS 144 EMGYTATLTR154 TTPTFFPKDI164 LTLRLDVMME174 TENRLHFTIK184 DPANRRYEVP 194 LETPSPLYSV211 EFSEEPFGVI221 VHRQLDGRVL231 LNTTVAPLFF241 ADQFLQLSTS 251 LPSQYITGLA261 EHLSPLMLST271 SWTRITLWNR281 DLAPTPGANL291 YGSHPFYLAL 301 EDGGSAHGVF311 LLNSNAMDVV321 LQPSPALSWR331 STGGILDVYI341 FLGPEPKSVV 351 QQYLDVVGYP361 FMPPYWGLGF371 HLCRWGYSST381 AITRQVVENM391 TRAHFPLDVQ 401 WNDLDYMDSR411 RDFTFNKDGF421 RDFPAMVQEL431 HQGGRRYMMI441 VDPAISSSGP 451 AGSYRPYDEG461 LRRGVFITNE471 TGQPLIGKVW481 PGSTAFPDFT491 NPTALAWWED 501 MVAEFHDQVP511 FDGMWIDMNE521 PSNFIRGSED531 GCPNNELENP541 PYVPGVVGGT 551 LQAATICASS561 HQFLSTHYNL571 HNLYGLTEAI581 ASHRALVKAR591 GTRPFVISRS 601 TFAGHGRYAG611 HWTGDVWSSW621 EQLASSVPEI631 LQFNLLGVPL641 VGADVCGFLG 651 NTSEELCVRW661 TQLGAFYPFM671 RNHNSLLSLP681 QEPYSFSEPA691 QQAMRKALTL 701 RYALLPHLYT711 LFHQAHVAGE721 TVARPLFLEF731 PKDSSTWTVD741 HQLLWGEALL 751 ITPVLQAGKA761 EVTGYFPLGT771 WYDLQTVPIE781 PREPAIHSEG802 QWVTLPAPLD 812 TINVHLRAGY822 IIPLQGPGLT832 TTESRQQPMA842 LAVALTKGGE852 ARGELFWDDG 862 ESLEVLERGA872 YTQVIFLARN882 NTIVNELVRV892 TSEGAGLQLQ902 KVTVLGVATA 912 PQQVLSNGVP922 VSNFTYSPDT932 KVLDICVSLL942 MGEQFLVSWC952 |
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Click to Show 3D Structure of This Binding Site
set background white;style ions nothing; color 8e8e8e;style chemicals nothing; select .BGC or .BGC2 or .BGC3 or :3BGC;style chemicals stick;color identity;select .A:93 or .A:96 or .A:97 or .A:98 or .A:99 or .A:126 or .A:282 or .A:376 or .A:404 or .A:405 or .A:441 or .A:481 or .A:516 or .A:518 or .A:519 or .A:600 or .A:613 or .A:616 or .A:645 or .A:649 or .A:672 or .A:674; color #f3c393; zoom selection;set surface opacity 0.5;set surface Van der Waals surface;set mode all
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ALA93
4.888
LYS96
4.053
ALA97
3.441
ILE98
2.919
THR99
4.839
TRP126
3.623
ASP282
4.672
TRP376
3.559
ASP404
2.521
LEU405
3.427
ILE441
3.383
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| Click to View More Binding Site Information of This Target and Ligand Pair | ||||||
| Ligand Name: Cysteine Sulfenic Acid | Ligand Info | |||||
| Structure Description | Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with 1-deoxynojirimycin | PDB:5NN5 | ||||
| Method | X-ray diffraction | Resolution | 2.00 Å | Mutation | No | [1] |
| PDB Sequence |
QCDVPPNSRF
90 DCAPDKAITQ100 EQCEARGCCY110 IPAKQGQPWC127 FFPPSYPSYK137 LENLSSSEMG 147 YTATLTRTTP157 TFFPKDILTL167 RLDVMMETEN177 RLHFTIKDPA187 NRRYEVPLET 197 APSPLYSVEF213 SEEPFGVIVH223 RQLDGRVLLN233 TTVAPLFFAD243 QFLQLSTSLP 253 SQYITGLAEH263 LSPLMLSTSW273 TRITLWNRDL283 APTPGANLYG293 SHPFYLALED 303 GGSAHGVFLL313 NSNAMDVVLQ323 PSPALSWRST333 GGILDVYIFL343 GPEPKSVVQQ 353 YLDVVGYPFM363 PPYWGLGFHL373 CRWGYSSTAI383 TRQVVENMTR393 AHFPLDVQWN 403 DLDYMDSRRD413 FTFNKDGFRD423 FPAMVQELHQ433 GGRRYMMIVD443 PAISSSGPAG 453 SYRPYDEGLR463 RGVFITNETG473 QPLIGKVWPG483 STAFPDFTNP493 TALAWWEDMV 503 AEFHDQVPFD513 GMWIDMNEPS523 NFIRGSEDGC533 PNNELENPPY543 VPGVVGGTLQ 553 AATICASSHQ563 FLSTHYNLHN573 LYGLTEAIAS583 HRALVKARGT593 RPFVISRSTF 603 AGHGRYAGHW613 TGDVWSSWEQ623 LASSVPEILQ633 FNLLGVPLVG643 ADVCGFLGNT 653 SEELCVRWTQ663 LGAFYPFMRN673 HNSLLSLPQE683 PYSFSEPAQQ693 AMRKALTLRY 703 ALLPHLYTLF713 HQAHVAGETV723 ARPLFLEFPK733 DSSTWTVDHQ743 LLWGEALLIT 753 PVLQAGKAEV763 TGYFPLGTWY773 DLQTVPIEEP796 AIHSEGQWVT806 LPAPLDTINV 816 HLRAGYIIPL826 QGPGLTTTES836 RQQPMALAVA846 LTKGGEARGE856 LFWDDGESLE 866 VLERGAYTQV876 IFLARNNTIV886 NELVRVTSEG896 AGLQLQKVTV906 LGVATAPQQV 916 LSNGVPVSNF926 TYSPDTKVLD936 IVSLLMGEQF947 LVSWC
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Click to Show 3D Structure of This Binding Site
set background white;style ions nothing; color 8e8e8e;style chemicals nothing; select .CSO or .CSO2 or .CSO3 or :3CSO;style chemicals stick;color identity;select .A:902 or .A:903 or .A:923 or .A:924 or .A:925 or .A:926 or .A:936 or .A:937 or .A:939 or .A:940; color #00ffc7; zoom selection;set surface opacity 0.5;set surface Van der Waals surface;set mode all
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| Click to View More Binding Site Information of This Target and Ligand Pair | ||||||
| Ligand Name: (2S,3R,4S,5S,6R)-6-(hydroxymethyl)oxane-2,3,4,5-tetrol | Ligand Info | |||||
| Structure Description | Crystal structure of human GAA | PDB:5KZW | ||||
| Method | X-ray diffraction | Resolution | 2.00 Å | Mutation | No | [2] |
| PDB Sequence |
PTQCDVPPNS
88 RFDCAPDKAI98 TQEQCEARGC108 CYIPAKQMGQ124 PWCFFPPSYP134 SYKLENLSSS 144 EMGYTATLTR154 TTPTFFPKDI164 LTLRLDVMME174 TENRLHFTIK184 DPANRRYEVP 194 LETPSPLYSV211 EFSEEPFGVI221 VHRQLDGRVL231 LNTTVAPLFF241 ADQFLQLSTS 251 LPSQYITGLA261 EHLSPLMLST271 SWTRITLWNR281 DLAPTPGANL291 YGSHPFYLAL 301 EDGGSAHGVF311 LLNSNAMDVV321 LQPSPALSWR331 STGGILDVYI341 FLGPEPKSVV 351 QQYLDVVGYP361 FMPPYWGLGF371 HLCRWGYSST381 AITRQVVENM391 TRAHFPLDVQ 401 WNDLDYMDSR411 RDFTFNKDGF421 RDFPAMVQEL431 HQGGRRYMMI441 VDPAISSSGP 451 AGSYRPYDEG461 LRRGVFITNE471 TGQPLIGKVW481 PGSTAFPDFT491 NPTALAWWED 501 MVAEFHDQVP511 FDGMWIDMNE521 PSNFIRGSED531 GCPNNELENP541 PYVPGVVGGT 551 LQAATICASS561 HQFLSTHYNL571 HNLYGLTEAI581 ASHRALVKAR591 GTRPFVISRS 601 TFAGHGRYAG611 HWTGDVWSSW621 EQLASSVPEI631 LQFNLLGVPL641 VGADVCGFLG 651 NTSEELCVRW661 TQLGAFYPFM671 RNHNSLLSLP681 QEPYSFSEPA691 QQAMRKALTL 701 RYALLPHLYT711 LFHQAHVAGE721 TVARPLFLEF731 PKDSSTWTVD741 HQLLWGEALL 751 ITPVLQAGKA761 EVTGYFPLGT771 WYDLQTVPIE781 PREPAIHSEG802 QWVTLPAPLD 812 TINVHLRAGY822 IIPLQGPGLT832 TTESRQQPMA842 LAVALTKGGE852 ARGELFWDDG 862 ESLEVLERGA872 YTQVIFLARN882 NTIVNELVRV892 TSEGAGLQLQ902 KVTVLGVATA 912 PQQVLSNGVP922 VSNFTYSPDT932 KVLDICVSLL942 MGEQFLVSWC952 |
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Click to Show 3D Structure of This Binding Site
set background white;style ions nothing; color 8e8e8e;style chemicals nothing; select .GLC or .GLC2 or .GLC3 or :3GLC;style chemicals stick;color identity;select .A:91 or .A:92 or .A:93 or .A:94 or .A:96 or .A:98 or .A:122 or .A:123 or .A:124 or .A:125 or .A:126 or .A:127 or .A:128 or .A:376 or .A:404 or .A:405 or .A:441 or .A:481 or .A:516 or .A:518 or .A:519 or .A:600 or .A:613 or .A:615 or .A:616 or .A:645 or .A:649 or .A:672 or .A:674; color #f3c393; zoom selection;set surface opacity 0.5;set surface Van der Waals surface;set mode all
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ASP91
2.722
CYS92
4.843
ALA93
3.012
PRO94
4.610
LYS96
4.935
ILE98
4.649
MET122
3.327
GLY123
2.842
GLN124
3.325
PRO125
3.252
TRP126
2.929
CYS127
2.784
PHE128
4.959
TRP376
3.732
ASP404
2.675
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| Click to View More Binding Site Information of This Target and Ligand Pair | ||||||
| References | Top | ||||
|---|---|---|---|---|---|
| REF 1 | Structure of human lysosomal acid Alpha-glucosidase-a guide for the treatment of Pompe disease. Nat Commun. 2017 Oct 24;8(1):1111. | ||||
| REF 2 | The structure of human GAA: structural basis of Pompe disease | ||||
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