PubMed:29674318
Annnotations
PubMed_ArguminSci
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| T1 | 143-275 | DRI_Background | denotes | ctosidases (EC 3.2.1.22) are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. |
| T2 | 276-326 | DRI_Background | denotes | α-Galactosidases take part in the turnover of cell |
| T3 | 358-434 | DRI_Background | denotes | in plants and in the lysosomal degradation of glycosphingolipids in animals. |
| T4 | 435-661 | DRI_Background | denotes | Deficiency of human α-galactosidase A (α-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3). |
| T5 | 730-894 | DRI_Background | denotes | An activity-based probe (ABP) covalently labeling the catalytic nucleophile of α-Gal A has been previously designed to study α-galactosidases for use in FD therapy. |
| T6 | 895-1050 | DRI_Approach | denotes | Here, we report that this ABP labels proteins in Nicotiana benthamiana leaf extracts, enabling the identification and biochemical characterization of an N. |
| T7 | 1079-1127 | DRI_Approach | denotes | we name here A1.1 (gene accession ID GJZM-1660). |
| T8 | 1128-1322 | DRI_Background | denotes | The transiently overexpressed and purified enzyme was a monomer lacking N-glycans and was active toward 4-methylumbelliferyl-α-d-galactopyranoside substrate (Km = 0.17 mm) over a broad pH range. |
| T9 | 1323-1518 | DRI_Background | denotes | A1.1 structural analysis by X-ray crystallography revealed marked similarities with human α-Gal A, even including A1.1's ability to hydrolyze Gb3 and lyso-Gb3, which are not endogenous in plants. |
| T10 | 1519-1691 | DRI_Approach | denotes | Of note, A1.1 uptake into FD fibroblasts reduced the elevated lyso-Gb3 levels in these cells, consistent with A1.1 delivery to lysosomes as revealed by confocal microscopy. |
| T11 | 1692-1952 | DRI_Outcome | denotes | The ease of production and the features of A1.1, such as stability over a broad pH range, combined with its capacity to degrade glycosphingolipid substrates, warrant further examination of its value as a potential therapeutic agent for ERT-based FD management. |
GlyCosmos6-Glycan-Motif-Image
| Id | Subject | Object | Predicate | Lexical cue | image |
|---|---|---|---|---|---|
| T1 | 225-234 | Glycan_Motif | denotes | galactose | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G68158BT|https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G65889KE |
| T3 | 343-357 | Glycan_Motif | denotes | galactomannans | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G93424OB |
| T4 | 593-614 | Glycan_Motif | denotes | globotriaosylceramide | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G00059MO |
| T5 | 616-619 | Glycan_Motif | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G00059MO |
| T6 | 656-659 | Glycan_Motif | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G00059MO |
| T7 | 1465-1468 | Glycan_Motif | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G00059MO |
| T8 | 1478-1481 | Glycan_Motif | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G00059MO |
| T9 | 1586-1589 | Glycan_Motif | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/0.10.0/png/binary/G00059MO |
GlyCosmos6-Glycan-Motif-Structure
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| T1 | 225-234 | https://glytoucan.org/Structures/Glycans/G65889KE | denotes | galactose |
| T2 | 225-234 | https://glytoucan.org/Structures/Glycans/G68158BT | denotes | galactose |
| T3 | 343-357 | https://glytoucan.org/Structures/Glycans/G93424OB | denotes | galactomannans |
| T4 | 593-614 | https://glytoucan.org/Structures/Glycans/G00059MO | denotes | globotriaosylceramide |
| T5 | 616-619 | https://glytoucan.org/Structures/Glycans/G00059MO | denotes | Gb3 |
| T6 | 656-659 | https://glytoucan.org/Structures/Glycans/G00059MO | denotes | Gb3 |
| T7 | 1465-1468 | https://glytoucan.org/Structures/Glycans/G00059MO | denotes | Gb3 |
| T8 | 1478-1481 | https://glytoucan.org/Structures/Glycans/G00059MO | denotes | Gb3 |
| T9 | 1586-1589 | https://glytoucan.org/Structures/Glycans/G00059MO | denotes | Gb3 |
sentences
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| T1 | 0-136 | Sentence | denotes | Nicotiana benthamiana α-galactosidase A1.1 can functionally complement human α-galactosidase A deficiency associated with Fabry disease. |
| T2 | 137-434 | Sentence | denotes | α-Galactosidases (EC 3.2.1.22) are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. α-Galactosidases take part in the turnover of cell wall-associated galactomannans in plants and in the lysosomal degradation of glycosphingolipids in animals. |
| T3 | 435-661 | Sentence | denotes | Deficiency of human α-galactosidase A (α-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3). |
| T4 | 662-729 | Sentence | denotes | Current management of FD involves enzyme-replacement therapy (ERT). |
| T5 | 730-894 | Sentence | denotes | An activity-based probe (ABP) covalently labeling the catalytic nucleophile of α-Gal A has been previously designed to study α-galactosidases for use in FD therapy. |
| T6 | 895-1127 | Sentence | denotes | Here, we report that this ABP labels proteins in Nicotiana benthamiana leaf extracts, enabling the identification and biochemical characterization of an N. benthamiana α-galactosidase we name here A1.1 (gene accession ID GJZM-1660). |
| T7 | 1128-1322 | Sentence | denotes | The transiently overexpressed and purified enzyme was a monomer lacking N-glycans and was active toward 4-methylumbelliferyl-α-d-galactopyranoside substrate (Km = 0.17 mm) over a broad pH range. |
| T8 | 1323-1518 | Sentence | denotes | A1.1 structural analysis by X-ray crystallography revealed marked similarities with human α-Gal A, even including A1.1's ability to hydrolyze Gb3 and lyso-Gb3, which are not endogenous in plants. |
| T9 | 1519-1691 | Sentence | denotes | Of note, A1.1 uptake into FD fibroblasts reduced the elevated lyso-Gb3 levels in these cells, consistent with A1.1 delivery to lysosomes as revealed by confocal microscopy. |
| T10 | 1692-1952 | Sentence | denotes | The ease of production and the features of A1.1, such as stability over a broad pH range, combined with its capacity to degrade glycosphingolipid substrates, warrant further examination of its value as a potential therapeutic agent for ERT-based FD management. |
Glycosmos6-GlycoEpitope
| Id | Subject | Object | Predicate | Lexical cue | glyco_epitope_db_id |
|---|---|---|---|---|---|
| T1 | 616-619 | GlycoEpitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T2 | 656-659 | GlycoEpitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T3 | 1465-1468 | GlycoEpitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T4 | 1478-1481 | GlycoEpitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T5 | 1586-1589 | GlycoEpitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
GlyCosmos15-Glycan
| Id | Subject | Object | Predicate | Lexical cue | image |
|---|---|---|---|---|---|
| T1 | 343-357 | Glycan | denotes | galactomannans | https://api.glycosmos.org/wurcs2image/latest/png/binary/G93424OB |
| T2 | 616-619 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T3 | 656-659 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T4 | 1465-1468 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T5 | 1478-1481 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T6 | 1586-1589 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
mondo_disease
| Id | Subject | Object | Predicate | Lexical cue | mondo_id |
|---|---|---|---|---|---|
| T1 | 122-135 | Disease | denotes | Fabry disease | http://purl.obolibrary.org/obo/MONDO_0010526 |
| T2 | 490-503 | Disease | denotes | Fabry disease | http://purl.obolibrary.org/obo/MONDO_0010526 |
| T3 | 505-507 | Disease | denotes | FD | http://purl.obolibrary.org/obo/MONDO_0010526 |
| T4 | 532-558 | Disease | denotes | lysosomal storage disorder | http://purl.obolibrary.org/obo/MONDO_0002561 |
| T5 | 684-686 | Disease | denotes | FD | http://purl.obolibrary.org/obo/MONDO_0010526 |
| T6 | 883-885 | Disease | denotes | FD | http://purl.obolibrary.org/obo/MONDO_0010526 |
| T7 | 1545-1547 | Disease | denotes | FD | http://purl.obolibrary.org/obo/MONDO_0010526 |
| T8 | 1938-1940 | Disease | denotes | FD | http://purl.obolibrary.org/obo/MONDO_0010526 |
Glycan-GlyCosmos
| Id | Subject | Object | Predicate | Lexical cue | image |
|---|---|---|---|---|---|
| T1 | 616-619 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T2 | 656-659 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T3 | 1465-1468 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T4 | 1478-1481 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
| T5 | 1586-1589 | Glycan | denotes | Gb3 | https://api.glycosmos.org/wurcs2image/latest/png/binary/G00059MO |
GlyCosmos-GlycoEpitope
| Id | Subject | Object | Predicate | Lexical cue | glycoepitope_id |
|---|---|---|---|---|---|
| T1 | 616-619 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T2 | 656-659 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T3 | 1465-1468 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T4 | 1478-1481 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T5 | 1586-1589 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
GlyCosmos15-CL
| Id | Subject | Object | Predicate | Lexical cue | cl_id |
|---|---|---|---|---|---|
| T1 | 1548-1559 | Cell | denotes | fibroblasts | http://purl.obolibrary.org/obo/CL:0000057 |
GlyCosmos15-UBERON
| Id | Subject | Object | Predicate | Lexical cue | uberon_id |
|---|---|---|---|---|---|
| T1 | 327-331 | Body_part | denotes | wall | http://purl.obolibrary.org/obo/UBERON_0000060 |
| T2 | 1548-1559 | Body_part | denotes | fibroblasts | http://purl.obolibrary.org/obo/CL_0000057 |
| T3 | 1646-1655 | Body_part | denotes | lysosomes | http://purl.obolibrary.org/obo/GO_0005764 |
GlyCosmos15-MONDO
| Id | Subject | Object | Predicate | Lexical cue | mondo_id |
|---|---|---|---|---|---|
| T1 | 122-135 | Disease | denotes | Fabry disease | MONDO:0010526 |
| T2 | 490-503 | Disease | denotes | Fabry disease | MONDO:0010526 |
| T3 | 505-507 | Disease | denotes | FD | MONDO:0010526 |
| T4 | 532-558 | Disease | denotes | lysosomal storage disorder | MONDO:0002561 |
| T5 | 684-686 | Disease | denotes | FD | MONDO:0010526 |
| T6 | 883-885 | Disease | denotes | FD | MONDO:0010526 |
| T7 | 1545-1547 | Disease | denotes | FD | MONDO:0010526 |
| T8 | 1938-1940 | Disease | denotes | FD | MONDO:0010526 |
GlyCosmos15-Taxon
| Id | Subject | Object | Predicate | Lexical cue | db_id |
|---|---|---|---|---|---|
| T1 | 0-21 | Organism | denotes | Nicotiana benthamiana | 4100 |
| T2 | 71-76 | Organism | denotes | human | 9606 |
| T3 | 449-454 | Organism | denotes | human | 9606 |
| T4 | 944-965 | Organism | denotes | Nicotiana benthamiana | 4100 |
| T5 | 1407-1412 | Organism | denotes | human | 9606 |
GlyCosmos15-GlycoEpitope
| Id | Subject | Object | Predicate | Lexical cue | glycoepitope_id |
|---|---|---|---|---|---|
| T1 | 616-619 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T2 | 656-659 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T3 | 1465-1468 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T4 | 1478-1481 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
| T5 | 1586-1589 | http://purl.jp/bio/12/glyco/glycan#Glycan_epitope | denotes | Gb3 | http://www.glycoepitope.jp/epitopes/EP0071 |
GlyCosmos15-Sentences
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| T1 | 0-136 | Sentence | denotes | Nicotiana benthamiana α-galactosidase A1.1 can functionally complement human α-galactosidase A deficiency associated with Fabry disease. |
| T2 | 137-434 | Sentence | denotes | α-Galactosidases (EC 3.2.1.22) are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. α-Galactosidases take part in the turnover of cell wall-associated galactomannans in plants and in the lysosomal degradation of glycosphingolipids in animals. |
| T3 | 435-661 | Sentence | denotes | Deficiency of human α-galactosidase A (α-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3). |
| T4 | 662-729 | Sentence | denotes | Current management of FD involves enzyme-replacement therapy (ERT). |
| T5 | 730-894 | Sentence | denotes | An activity-based probe (ABP) covalently labeling the catalytic nucleophile of α-Gal A has been previously designed to study α-galactosidases for use in FD therapy. |
| T6 | 895-1127 | Sentence | denotes | Here, we report that this ABP labels proteins in Nicotiana benthamiana leaf extracts, enabling the identification and biochemical characterization of an N. benthamiana α-galactosidase we name here A1.1 (gene accession ID GJZM-1660). |
| T7 | 1128-1322 | Sentence | denotes | The transiently overexpressed and purified enzyme was a monomer lacking N-glycans and was active toward 4-methylumbelliferyl-α-d-galactopyranoside substrate (Km = 0.17 mm) over a broad pH range. |
| T8 | 1323-1518 | Sentence | denotes | A1.1 structural analysis by X-ray crystallography revealed marked similarities with human α-Gal A, even including A1.1's ability to hydrolyze Gb3 and lyso-Gb3, which are not endogenous in plants. |
| T9 | 1519-1691 | Sentence | denotes | Of note, A1.1 uptake into FD fibroblasts reduced the elevated lyso-Gb3 levels in these cells, consistent with A1.1 delivery to lysosomes as revealed by confocal microscopy. |
| T10 | 1692-1952 | Sentence | denotes | The ease of production and the features of A1.1, such as stability over a broad pH range, combined with its capacity to degrade glycosphingolipid substrates, warrant further examination of its value as a potential therapeutic agent for ERT-based FD management. |
GlyCosmos15-FMA
| Id | Subject | Object | Predicate | Lexical cue | db_id |
|---|---|---|---|---|---|
| T1 | 379-388 | Body_part | denotes | lysosomal | FMA:63836 |
| T2 | 532-541 | Body_part | denotes | lysosomal | FMA:63836 |
| T3 | 1548-1559 | Body_part | denotes | fibroblasts | FMA:63877 |
| T4 | 1646-1655 | Body_part | denotes | lysosomes | FMA:63836 |
NCBITAXON
| Id | Subject | Object | Predicate | Lexical cue | db_id |
|---|---|---|---|---|---|
| T1 | 0-21 | OrganismTaxon | denotes | Nicotiana benthamiana | 4100 |
| T2 | 71-76 | OrganismTaxon | denotes | human | 9606 |
| T3 | 449-454 | OrganismTaxon | denotes | human | 9606 |
| T4 | 944-965 | OrganismTaxon | denotes | Nicotiana benthamiana | 4100 |
| T5 | 1407-1412 | OrganismTaxon | denotes | human | 9606 |
Anatomy-UBERON
| Id | Subject | Object | Predicate | Lexical cue | uberon_id |
|---|---|---|---|---|---|
| T1 | 327-331 | Body_part | denotes | wall | http://purl.obolibrary.org/obo/UBERON_0000060 |
| T2 | 1548-1559 | Body_part | denotes | fibroblasts | http://purl.obolibrary.org/obo/CL_0000057 |
| T3 | 1646-1655 | Body_part | denotes | lysosomes | http://purl.obolibrary.org/obo/GO_0005764 |
CL-cell
| Id | Subject | Object | Predicate | Lexical cue | cl_id |
|---|---|---|---|---|---|
| T1 | 1548-1559 | Cell | denotes | fibroblasts | http://purl.obolibrary.org/obo/CL:0000057 |