DB code: D00864
| RLCP classification | 1.30.35885.972 : Hydrolysis | |
|---|---|---|
| CATH domain | 3.20.20.80 : TIM Barrel | Catalytic domain |
| 2.60.40.1180 : Immunoglobulin-like | ||
| E.C. | 3.2.1.45 | |
| CSA | 2f61 | |
| M-CSA | 2f61 | |
| MACiE | ||
| CATH domain | Related DB codes (homologues) |
|---|---|
| 2.60.40.1180 : Immunoglobulin-like | M00113 T00307 D00165 D00176 D00664 D00665 D00863 M00112 M00193 M00314 T00057 T00062 T00067 |
| 3.20.20.80 : TIM Barrel | S00202 S00210 S00748 S00906 S00907 S00911 S00912 S00915 M00134 M00160 D00479 S00204 S00205 S00206 S00207 S00203 S00208 S00209 S00211 S00213 S00214 M00113 T00307 D00165 D00166 D00169 D00176 D00501 D00502 D00503 D00844 D00861 M00026 M00112 M00193 M00346 T00057 T00062 T00063 T00066 T00067 |
| Uniprot Enzyme Name | UniprotKB | Protein name | Synonyms | RefSeq | CAZy | Pfam |
|---|---|---|---|---|---|
| Q9BDT0 |
Glucosylceramidase
|
EC
3.2.1.45
Acid beta-glucosidase Beta-glucocerebrosidase D-glucosyl-N-acylsphingosine glucohydrolase |
NP_001008997.1
(Protein)
NM_001008997.1 (DNA/RNA sequence) |
GH30
(Glycoside Hydrolase Family 30)
|
PF02055
(Glyco_hydro_30)
[Graphical View] |
| P04062 |
Glucosylceramidase
|
EC
3.2.1.45
Acid beta-glucosidase Alglucerase Beta-glucocerebrosidase D-glucosyl-N-acylsphingosine glucohydrolase Imiglucerase |
NP_000148.2
(Protein)
NM_000157.3 (DNA/RNA sequence) NP_001005741.1 (Protein) NM_001005741.2 (DNA/RNA sequence) NP_001005742.1 (Protein) NM_001005742.2 (DNA/RNA sequence) |
GH30
(Glycoside Hydrolase Family 30)
|
PF02055
(Glyco_hydro_30)
[Graphical View] |
| KEGG enzyme name |
|---|
|
Glucosylceramidase
Psychosine hydrolase Glucosphingosine glucosylhydrolase GlcCer-beta-glucosidase beta-D-Glucocerebrosidase Glucosylcerebrosidase beta-Glucosylceramidase Ceramide glucosidase Glucocerebrosidase Glucosylsphingosine beta-glucosidase Glucosylsphingosine beta-D-glucosidase |
| UniprotKB: Accession Number | Entry name | Activity | Subunit | Subcellular location | Cofactor |
|---|---|---|---|---|---|
| Q9BDT0 | GLCM_PANTR | D-glucosyl-N-acylsphingosine + H(2)O = D-glucose + N-acylsphingosine. | Interacts with SCARB2 (By similarity). | Lysosome membrane, Peripheral membrane protein (By similarity). Note=Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (By similarity). | |
| P04062 | GLCM_HUMAN | D-glucosyl-N-acylsphingosine + H(2)O = D-glucose + N-acylsphingosine. | Interacts with saposin-C. | Lysosome membrane, Peripheral membrane protein, Lumenal side. Note=Interaction with saposin-C promotes membrane association. |
| KEGG Pathways | Map code | Pathways | E.C. |
|---|---|---|
| MAP00600 | Sphingolipid metabolism | |
| MAP01032 | Glycan structures - degradation |
| Compound table | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Substrates | Products | Intermediates | ||||||||
| KEGG-id | C01190 | C00001 | C00031 | C00195 | I00122 | |||||
| E.C. | ||||||||||
| Compound | D-glucosyl-N-acylsphingosine | H2O | D-glucose | N-acylsphingosine | Peptidyl-Glu-Glucose | |||||
| Type | amide group,carbohydrate,lipid | H2O | carbohydrate | amide group,carbohydrate,lipid | ||||||
| ChEBI |
15377 15377 |
4167 4167 |
||||||||
| PubChem |
22247451 962 22247451 962 |
5793 5793 |
||||||||
| 2vt0A01 |
|
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Unbound | Unbound | Unbound | Intermediate-analogue:CBU | |
| 2vt0B01 |
|
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Unbound | Unbound | Unbound | Intermediate-analogue:CBU | |
| 1ogsA01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 1ogsB01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 1y7vA01 |
|
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Unbound | Unbound | Unbound | Intermediate-analogue:INS | |
| 1y7vB01 |
|
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Unbound | Unbound | Unbound | Intermediate-analogue:INS | |
| 2f61A01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2f61B01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2j25A01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2j25B01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxA01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxB01 |
|
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Unbound | Analogue:IFM | Unbound | Unbound | |
| 2nsxC01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxD01 |
|
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Unbound | Analogue:IFM | Unbound | Unbound | |
| 2nt0A01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0B01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0C01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0D01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1A01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1B01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1C01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1D01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3dA01 |
|
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Analogue:NBV | Unbound | Unbound | Unbound | |
| 2v3dB01 |
|
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|
Analogue:NBV | Unbound | Unbound | Unbound | |
| 2v3eA01 |
|
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|
Analogue:NND | Unbound | Unbound | Unbound | |
| 2v3eB01 |
|
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|
Analogue:NND | Unbound | Unbound | Unbound | |
| 2v3fA01 |
|
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3fB01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 2wcgA01 |
|
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|
Unbound | Analogue:MT5 | Unbound | Unbound | |
| 2wcgB01 |
|
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|
Unbound | Analogue:MT5 | Unbound | Unbound | |
| 2wklA01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 2wklB01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 2xwdA01 |
|
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|
Unbound | Analogue:LGS | Unbound | Unbound | |
| 2xwdB01 |
|
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|
Unbound | Analogue:LGS | Unbound | Unbound | |
| 2xweA01 |
|
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|
Analogue:AMF | Unbound | Unbound | Unbound | |
| 2xweB01 |
|
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|
Analogue:AMF | Unbound | Unbound | Unbound | |
| 3gxdA01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 3gxdB01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 3gxdC01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxdD01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxfA01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 3gxfB01 |
|
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Unbound | Analogue:IFM | Unbound | Unbound | |
| 3gxfC01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxfD01 |
|
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|
Unbound | Analogue:IFM | Unbound | Unbound | |
| 3gxiA01 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 3gxiB01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxiC01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxiD01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxmA01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxmB01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxmC01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxmD01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3ke0A01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3ke0B01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3kehA01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3kehB01 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2vt0A02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2vt0B02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 1ogsA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 1ogsB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 1y7vA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 1y7vB02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2f61A02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2f61B02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2j25A02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2j25B02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxA02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxB02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxC02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nsxD02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0A02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0B02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0C02 |
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Unbound | Unbound | Unbound | Unbound | |
| 2nt0D02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1A02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1B02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1C02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2nt1D02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3dA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3dB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3eA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3eB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3fA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2v3fB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2wcgA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2wcgB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2wklA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2wklB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2xwdA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2xwdB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2xweA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 2xweB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxdA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxdB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxdC02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxdD02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxfA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxfB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxfC02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxfD02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxiA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxiB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxiC02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxiD02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxmA02 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 3gxmB02 |
|
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|
Unbound | Unbound | Unbound | Unbound | |
| 3gxmC02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3gxmD02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3ke0A02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3ke0B02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3kehA02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| 3kehB02 |
|
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Unbound | Unbound | Unbound | Unbound | |
| Reference for Active-site residues | ||
|---|---|---|
| resource | references | E.C. |
| literature [5], [7] | ||
| Active-site residues | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| PDB | Catalytic residues | Cofactor-binding residues | Modified residues | Main-chain involved in catalysis | Comment | |||||
| 2vt0A01 |
|
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|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2vt0B01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 1ogsA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 1ogsB01 |
|
|
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|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 1y7vA01 |
|
|
|
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 1y7vB01 |
|
|
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|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2f61A01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2f61B01 |
|
|
|
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2j25A01 |
|
|
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|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2j25B01 |
|
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|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nsxA01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nsxB01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nsxC01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nsxD01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt0A01 |
|
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|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt0B01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt0C01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt0D01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt1A01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt1B01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt1C01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2nt1D01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2v3dA01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2v3dB01 |
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2v3eA01 |
|
|
|
|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2v3eB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2v3fA01 |
|
|
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|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2v3fB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2wcgA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2wcgB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2wklA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2wklB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2xwdA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2xwdB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2xweA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 2xweB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxdA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxdB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxdC01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxdD01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxfA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxfB01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxfC01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxfD01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxiA01 |
|
|
|
|
|
ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxiB01 |
|
|
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|
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxiC01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxiD01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxmA01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxmB01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxmC01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3gxmD01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | ||||
| 3ke0A01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | mutant N370S | |||
| 3ke0B01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | mutant N370S | |||
| 3kehA01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | mutant N370S | |||
| 3kehB01 |
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ARG 120;ASN 234;GLU 235;HIS 311;TYR 313;GLU 340 | mutant N370S | |||
| 2vt0A02 |
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| 2vt0B02 |
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| 1ogsA02 |
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| 1ogsB02 |
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| 1y7vA02 |
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| 1y7vB02 |
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| 2f61A02 |
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| 2f61B02 |
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| 2j25A02 |
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| 2j25B02 |
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| 2nsxA02 |
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| 2nsxB02 |
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| 2nsxC02 |
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| 2nsxD02 |
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| 2nt0A02 |
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| 2nt0B02 |
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| 2nt0C02 |
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| 2nt0D02 |
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| 2nt1A02 |
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| 2nt1B02 |
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| 2nt1C02 |
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| 2nt1D02 |
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| 2v3dA02 |
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| 2v3dB02 |
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| 2v3eA02 |
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| 2v3eB02 |
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| 2v3fA02 |
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| 2v3fB02 |
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| 2wcgA02 |
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| 2wcgB02 |
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| 2wklA02 |
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| 2wklB02 |
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| 2xwdA02 |
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| 2xwdB02 |
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| 2xweA02 |
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| 2xweB02 |
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| 3gxdA02 |
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| 3gxdB02 |
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| 3gxdC02 |
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| 3gxdD02 |
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| 3gxfA02 |
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| 3gxfB02 |
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| 3gxfC02 |
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| 3gxfD02 |
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| 3gxiA02 |
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| 3gxiB02 |
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| 3gxiC02 |
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| 3gxiD02 |
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| 3gxmA02 |
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| 3gxmB02 |
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| 3gxmC02 |
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| 3gxmD02 |
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| 3ke0A02 |
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| 3ke0B02 |
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| 3kehA02 |
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| 3kehB02 |
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| References for Catalytic Mechanism | ||
|---|---|---|
| References | Sections | No. of steps in catalysis |
|
[3]
|
Fig.1, p.886, p.890 | |
|
[6]
|
Fig.1, p.853 | |
|
[12]
|
p.4242 | |
|
[14]
|
Fig.6, p.29055-29057 | |
| References | |
|---|---|
| [1] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 3927728 |
| Journal | Am J Med Genet |
| Year | 1985 |
| Volume | 21 |
| Pages | 529-49 |
| Authors | Grabowski GA, Goldblatt J, Dinur T, Kruse J, Svennerholm L, Gatt S, Desnick RJ |
| Title | Genetic heterogeneity in Gaucher disease: physicokinetic and immunologic studies of the residual enzyme in cultured fibroblasts from non-neuronopathic and neuronopathic patients. |
| Related PDB | |
| Related UniProtKB | |
| [2] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 1714449 |
| Journal | J Biol Chem |
| Year | 1991 |
| Volume | 266 |
| Pages | 15021-7 |
| Authors | Fabbro D, Grabowski GA |
| Title |
Human acid beta-glucosidase. |
| Related PDB | |
| Related UniProtKB | |
| [3] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 7712292 |
| Journal | Curr Opin Struct Biol |
| Year | 1994 |
| Volume | 4 |
| Pages | 885-92 |
| Authors | McCarter JD, Withers SG |
| Title | Mechanisms of enzymatic glycoside hydrolysis. |
| Related PDB | |
| Related UniProtKB | |
| [4] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 8294487 |
| Journal | J Biol Chem |
| Year | 1994 |
| Volume | 269 |
| Pages | 2283-91 |
| Authors | Grace ME, Newman KM, Scheinker V, Berg-Fussman A, Grabowski GA |
| Title | Analysis of human acid beta-glucosidase by site-directed mutagenesis and heterologous expression. |
| Related PDB | |
| Related UniProtKB | |
| [5] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 7908905 |
| Journal | J Biol Chem |
| Year | 1994 |
| Volume | 269 |
| Pages | 10975-8 |
| Authors | Miao S, McCarter JD, Grace ME, Grabowski GA, Aebersold R, Withers SG |
| Title | Identification of Glu340 as the active-site nucleophile in human glucocerebrosidase by use of electrospray tandem mass spectrometry. |
| Related PDB | |
| Related UniProtKB | P04062 |
| [6] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 8535779 |
| Journal | Structure |
| Year | 1995 |
| Volume | 3 |
| Pages | 853-9 |
| Authors | Davies G, Henrissat B |
| Title | Structures and mechanisms of glycosyl hydrolases. |
| Related PDB | |
| Related UniProtKB | |
| [7] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 11087714 |
| Journal | Glycobiology |
| Year | 2000 |
| Volume | 10 |
| Pages | 1217-24 |
| Authors | Fabrega S, Durand P, Codogno P, Bauvy C, Delomenie C, Henrissat B, Martin BM, McKinney C, Ginns EI, Mornon JP, Lehn P |
| Title | Human glucocerebrosidase: heterologous expression of active site mutants in murine null cells. |
| Related PDB | |
| Related UniProtKB | |
| [8] | |
| Resource | |
| Comments |
X-RAY CRYSTALLOGRAPHY (2.0 ANGSTROMS) OF 40-536, |
| Medline ID | |
| PubMed ID | 12792654 |
| Journal | EMBO Rep |
| Year | 2003 |
| Volume | 4 |
| Pages | 704-9 |
| Authors | Dvir H, Harel M, McCarthy AA, Toker L, Silman I, Futerman AH, Sussman JL |
| Title |
X-ray structure of human acid-beta-glucosidase, |
| Related PDB | 1ogs |
| Related UniProtKB | P04062 |
| [9] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 15019270 |
| Journal | Trends Pharmacol Sci |
| Year | 2004 |
| Volume | 25 |
| Pages | 147-51 |
| Authors | Futerman AH, Sussman JL, Horowitz M, Silman I, Zimran A |
| Title | New directions in the treatment of Gaucher disease. |
| Related PDB | |
| Related UniProtKB | |
| [10] | |
| Resource | |
| Comments |
X-RAY CRYSTALLOGRAPHY (2.4 ANGSTROMS) OF 40-536 IN COMPLEX WITH SYNTHETIC INHIBITOR, |
| Medline ID | |
| PubMed ID | 15817452 |
| Journal | J Biol Chem |
| Year | 2005 |
| Volume | 280 |
| Pages | 23815-9 |
| Authors | Premkumar L, Sawkar AR, Boldin-Adamsky S, Toker L, Silman I, Kelly JW, Futerman AH, Sussman JL |
| Title |
X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. |
| Related PDB | 1y7v |
| Related UniProtKB | P04062 |
| [11] | |
| Resource | |
| Comments |
X-RAY CRYSTALLOGRAPHY (2.9 ANGSTROMS) OF 40-536, |
| Medline ID | |
| PubMed ID | 17139081 |
| Journal | Acta Crystallogr D Biol Crystallogr |
| Year | 2006 |
| Volume | 62 |
| Pages | 1458-65 |
| Authors | Brumshtein B, Wormald MR, Silman I, Futerman AH, Sussman JL |
| Title |
Structural comparison of differently glycosylated forms of acid-beta-glucosidase, |
| Related PDB | 2j25 |
| Related UniProtKB | P04062 |
| [12] | |
| Resource | |
| Comments |
X-RAY CRYSTALLOGRAPHY (2.5 ANGSTROMS) OF 40-536, |
| Medline ID | |
| PubMed ID | 16293621 |
| Journal | J Biol Chem |
| Year | 2006 |
| Volume | 281 |
| Pages | 4242-53 |
| Authors | Liou B, Kazimierczuk A, Zhang M, Scott CR, Hegde RS, Grabowski GA |
| Title | Analyses of variant acid beta-glucosidases: effects of Gaucher disease mutations. |
| Related PDB | 2f61 |
| Related UniProtKB | P04062 |
| [13] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 17894779 |
| Journal | FEBS J |
| Year | 2007 |
| Volume | 274 |
| Pages | 4944-50 |
| Authors | Yu Z, Sawkar AR, Kelly JW |
| Title | Pharmacologic chaperoning as a strategy to treat Gaucher disease. |
| Related PDB | |
| Related UniProtKB | |
| [14] | |
| Resource | |
| Comments | X-RAY CRYSTALLOGRAPHY |
| Medline ID | |
| PubMed ID | 17666401 |
| Journal | J Biol Chem |
| Year | 2007 |
| Volume | 282 |
| Pages | 29052-8 |
| Authors | Brumshtein B, Greenblatt HM, Butters TD, Shaaltiel Y, Aviezer D, Silman I, Futerman AH, Sussman JL |
| Title | Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease. |
| Related PDB | 2v3e 2v3d |
| Related UniProtKB | |
| [15] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 17713797 |
| Journal | J Mol Model |
| Year | 2007 |
| Volume | 13 |
| Pages | 1133-9 |
| Authors | Zubrzycki IZ, Borcz A, Wiacek M, Hagner W |
| Title |
The studies on substrate, |
| Related PDB | |
| Related UniProtKB | |
| [16] | |
| Resource | |
| Comments |
X-RAY CRYSTALLOGRAPHY (1.8 ANGSTROMS) OF 40-536 IN COMPLEXES WITH ISOFAGOMINE, |
| Medline ID | |
| PubMed ID | 17187079 |
| Journal | Nat Chem Biol |
| Year | 2007 |
| Volume | 3 |
| Pages | 101-7 |
| Authors | Lieberman RL, Wustman BA, Huertas P, Powe AC Jr, Pine CW, Khanna R, Schlossmacher MG, Ringe D, Petsko GA |
| Title | Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease. |
| Related PDB | 2nt0 2nt1 2nsx |
| Related UniProtKB | P04062 |
| [17] | |
| Resource | |
| Comments | X-RAY CRYSTALLOGRAPHY |
| Medline ID | |
| PubMed ID | 17524049 |
| Journal | Plant Biotechnol J |
| Year | 2007 |
| Volume | 5 |
| Pages | 579-90 |
| Authors | Shaaltiel Y, Bartfeld D, Hashmueli S, Baum G, Brill-Almon E, Galili G, Dym O, Boldin-Adamsky SA, Silman I, Sussman JL, Futerman AH, Aviezer D |
| Title | Production of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell system. |
| Related PDB | 2v3f |
| Related UniProtKB | |
| [18] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 18783340 |
| Journal | Biol Chem |
| Year | 2008 |
| Volume | 389 |
| Pages | 1361-9 |
| Authors | Kacher Y, Brumshtein B, Boldin-Adamsky S, Toker L, Shainskaya A, Silman I, Sussman JL, Futerman AH |
| Title | Acid beta-glucosidase: insights from structural analysis and relevance to Gaucher disease therapy. |
| Related PDB | 2vt0 |
| Related UniProtKB | |
| [19] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 19374450 |
| Journal | Biochemistry |
| Year | 2009 |
| Volume | 48 |
| Pages | 4816-27 |
| Authors | Lieberman RL, D'aquino JA, Ringe D, Petsko GA |
| Title | Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability. |
| Related PDB | 3gxd 3gxf 3gxi 3gxm |
| Related UniProtKB | |
| [20] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 19437524 |
| Journal | Chembiochem |
| Year | 2009 |
| Volume | 10 |
| Pages | 1480-5 |
| Authors | Brumshtein B, Aguilar-Moncayo M, Garc?a-Moreno MI, Ortiz Mellet C, Garc?a Fern?ndez JM, Silman I, Shaaltiel Y, Aviezer D, Sussman JL, Futerman AH |
| Title |
6-Amino-6-deoxy-5,6-di-N-(N'-octyliminomethylidene)nojirimycin: synthesis, |
| Related PDB | 2wcg |
| Related UniProtKB | |
| [21] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 20980263 |
| Journal | J Biol Chem |
| Year | 2011 |
| Volume | 286 |
| Pages | 299-308 |
| Authors | Wei RR, Hughes H, Boucher S, Bird JJ, Guziewicz N, Van Patten SM, Qiu H, Pan CQ, Edmunds T |
| Title | X-ray and biochemical analysis of N370S mutant human acid ¦Â-glucosidase. |
| Related PDB | 3ke0 3keh |
| Related UniProtKB | |
| [22] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 21483943 |
| Journal | Org Biomol Chem |
| Year | 2011 |
| Volume | 9 |
| Pages | 4160-7 |
| Authors | Brumshtein B, Aguilar-Moncayo M, Benito JM, Garc?a Fernandez JM, Silman I, Shaaltiel Y, Aviezer D, Sussman JL, Futerman AH, Ortiz Mellet C |
| Title | Cyclodextrin-mediated crystallization of acid ¦Â-glucosidase in complex with amphiphilic bicyclic nojirimycin analogues. |
| Related PDB | 2xwd 2xwe |
| Related UniProtKB | |
| Comments |
|---|
|
The reaction may proceeds as follows:
This enzyme belongs to glycosidase family-30, This enzyme seems to be distantly related to beta-xylosidase (EC 3.2.1.37; D00844 in EzCatDB) from glycosidase family-39, (0) Arg120 and Tyr313 may modulate the activity of catalytic nucleophile, (1) Glu235 acts as a general acid to protonate the leaving oxygen, (2) Glu340 makes a nucleophilic attack on C1 atom of substrate, (3) Glu235 acts as a general base to deprotonate a water molecule, (4) The activated water makes a nucleophilic attack on the C1 atom of the covalent intermediate. |
| Created | Updated |
|---|---|
| 2008-05-13 | 2012-02-14 |