PubMed:25054547
Annnotations
test-210614
Id | Subject | Object | Predicate | Lexical cue | proteinmutation |
---|---|---|---|---|---|
25054547_0 | 1884-1894 | ProteinMutation | denotes | p70Gly-p.A | rs1799807 |
25054547_1 | 1895-1906 | ProteinMutation | denotes | a539Thr tet | rs1803274 |
25054547_2 | 1157-1167 | ProteinMutation | denotes | p70Gly-p.A | rs1799807 |
25054547_3 | 1168-1179 | ProteinMutation | denotes | a539Thr BCh | rs1803274 |
PubTator4TogoVar
Id | Subject | Object | Predicate | Lexical cue | proteinmutation |
---|---|---|---|---|---|
25054547_0 | 1884-1894 | ProteinMutation | denotes | p70Gly-p.A | rs1799807 |
25054547_1 | 1895-1906 | ProteinMutation | denotes | a539Thr tet | rs1803274 |
25054547_2 | 1157-1167 | ProteinMutation | denotes | p70Gly-p.A | rs1799807 |
25054547_3 | 1168-1179 | ProteinMutation | denotes | a539Thr BCh | rs1803274 |
LitCoin-sentences
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 0-142 | Sentence | denotes | Characterization of a novel BCHE "silent" allele: point mutation (p.Val204Asp) causes loss of activity and prolonged apnea with suxamethonium. |
T2 | 143-324 | Sentence | denotes | Butyrylcholinesterase deficiency is characterized by prolonged apnea after the use of muscle relaxants (suxamethonium or mivacurium) in patients who have mutations in the BCHE gene. |
T3 | 325-492 | Sentence | denotes | Here, we report a case of prolonged neuromuscular block after administration of suxamethonium leading to the discovery of a novel BCHE variant (c.695T>A, p.Val204Asp). |
T4 | 493-666 | Sentence | denotes | Inhibition studies, kinetic analysis and molecular dynamics were undertaken to understand how this mutation disrupts the catalytic triad and determines a "silent" phenotype. |
T5 | 667-862 | Sentence | denotes | Low activity of patient plasma butyrylcholinesterase with butyrylthiocholine (BTC) and benzoylcholine, and values of dibucaine and fluoride numbers fit with heterozygous atypical silent genotype. |
T6 | 863-1107 | Sentence | denotes | Electrophoretic analysis of plasma BChE of the proband and his mother showed that patient has a reduced amount of tetrameric enzyme in plasma and that minor fast-moving BChE components: monomer, dimer, and monomer-albumin conjugate are missing. |
T7 | 1108-1243 | Sentence | denotes | Kinetic analysis showed that the p.Val204Asp/p.Asp70Gly-p.Ala539Thr BChE displays a pure Michaelian behavior with BTC as the substrate. |
T8 | 1244-1423 | Sentence | denotes | Both catalytic parameters Km = 265 µM for BTC, two times higher than that of the atypical enzyme, and a low Vmax are consistent with the absence of activity against suxamethonium. |
T9 | 1424-1734 | Sentence | denotes | Molecular dynamic (MD) simulations showed that the overall effect of the mutation p.Val204Asp is disruption of hydrogen bonding between Gln223 and Glu441, leading Ser198 and His438 to move away from each other with subsequent disruption of the catalytic triad functionality regardless of the type of substrate. |
T10 | 1735-1823 | Sentence | denotes | MD also showed that the enzyme volume is increased, suggesting a pre-denaturation state. |
T11 | 1824-1996 | Sentence | denotes | This fits with the reduced concentration of p.Ala204Asp/p.Asp70Gly-p.Ala539Thr tetrameric enzyme in the plasma and non-detectable fast moving-bands on electrophoresis gels. |
LitCoin-entities
Id | Subject | Object | Predicate | Lexical cue | db_id |
---|---|---|---|---|---|
10153 | 28-32 | GeneOrGeneProduct | denotes | BCHE | NCBIGene:590 |
10154 | 66-77 | SequenceVariant | denotes | p.Val204Asp | p|SUB|V|204|D |
10155 | 117-122 | DiseaseOrPhenotypicFeature | denotes | apnea | MESH:D001049 |
10156 | 128-141 | ChemicalEntity | denotes | suxamethonium | MESH:D013390 |
10157 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | MESH:C537417 |
10158 | 206-211 | DiseaseOrPhenotypicFeature | denotes | apnea | MESH:D001049 |
10159 | 229-245 | ChemicalEntity | denotes | muscle relaxants | |
10160 | 247-260 | ChemicalEntity | denotes | suxamethonium | MESH:D013390 |
10161 | 264-274 | ChemicalEntity | denotes | mivacurium | MESH:D000077590 |
10162 | 279-287 | OrganismTaxon | denotes | patients | NCBITaxon:9606 |
10163 | 314-318 | GeneOrGeneProduct | denotes | BCHE | NCBIGene:590 |
10164 | 361-380 | DiseaseOrPhenotypicFeature | denotes | neuromuscular block | MESH:D055191 |
10165 | 405-418 | ChemicalEntity | denotes | suxamethonium | MESH:D013390 |
10166 | 455-459 | GeneOrGeneProduct | denotes | BCHE | NCBIGene:590 |
10167 | 469-477 | SequenceVariant | denotes | c.695T>A | c|SUB|T|695|A |
10168 | 479-490 | SequenceVariant | denotes | p.Val204Asp | p|SUB|V|204|D |
10169 | 683-690 | OrganismTaxon | denotes | patient | NCBITaxon:9606 |
10170 | 698-719 | GeneOrGeneProduct | denotes | butyrylcholinesterase | NCBIGene:590 |
10171 | 725-743 | ChemicalEntity | denotes | butyrylthiocholine | MESH:D002092 |
10172 | 745-748 | ChemicalEntity | denotes | BTC | MESH:D002092 |
10173 | 754-768 | ChemicalEntity | denotes | benzoylcholine | MESH:D001588 |
10174 | 784-793 | ChemicalEntity | denotes | dibucaine | MESH:D003992 |
10175 | 798-806 | ChemicalEntity | denotes | fluoride | MESH:D005459 |
10176 | 898-902 | GeneOrGeneProduct | denotes | BChE | NCBIGene:590 |
10177 | 945-952 | OrganismTaxon | denotes | patient | NCBITaxon:9606 |
10178 | 1032-1036 | GeneOrGeneProduct | denotes | BChE | NCBIGene:590 |
10179 | 1141-1152 | SequenceVariant | denotes | p.Val204Asp | p|SUB|V|204|D |
10180 | 1153-1163 | SequenceVariant | denotes | p.Asp70Gly | DBSNP:rs1799807 |
10181 | 1164-1175 | SequenceVariant | denotes | p.Ala539Thr | DBSNP:rs1803274 |
10182 | 1176-1180 | GeneOrGeneProduct | denotes | BChE | NCBIGene:590 |
10183 | 1222-1225 | ChemicalEntity | denotes | BTC | MESH:D002092 |
10184 | 1286-1289 | ChemicalEntity | denotes | BTC | MESH:D002092 |
10185 | 1409-1422 | ChemicalEntity | denotes | suxamethonium | MESH:D013390 |
10186 | 1506-1517 | SequenceVariant | denotes | p.Val204Asp | p|SUB|V|204|D |
10187 | 1560-1566 | SequenceVariant | denotes | Gln223 | p|Allele|Q|223 |
10188 | 1571-1577 | SequenceVariant | denotes | Glu441 | p|Allele|E|2441 |
10189 | 1587-1593 | SequenceVariant | denotes | Ser198 | p|Allele|S|198 |
10190 | 1598-1604 | SequenceVariant | denotes | His438 | p|Allele|H|438 |
10191 | 1868-1879 | SequenceVariant | denotes | p.Ala204Asp | p|SUB|A|204|D |
10192 | 1880-1890 | SequenceVariant | denotes | p.Asp70Gly | DBSNP:rs1799807 |
10193 | 1891-1902 | SequenceVariant | denotes | p.Ala539Thr | DBSNP:rs1803274 |
LitCoin_Mondo
Id | Subject | Object | Predicate | Lexical cue | mondo_id |
---|---|---|---|---|---|
T1 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | 0015270 |
LitCoin-SeqVar
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 66-77 | SequenceVariant | denotes | p.Val204Asp |
T2 | 469-477 | SequenceVariant | denotes | c.695T>A |
T3 | 479-490 | SequenceVariant | denotes | p.Val204Asp |
T4 | 1141-1152 | SequenceVariant | denotes | p.Val204Asp |
T5 | 1153-1163 | SequenceVariant | denotes | p.Asp70Gly |
T6 | 1164-1175 | SequenceVariant | denotes | p.Ala539Thr |
T7 | 1506-1517 | SequenceVariant | denotes | p.Val204Asp |
T8 | 1868-1879 | SequenceVariant | denotes | p.Ala204Asp |
T9 | 1880-1890 | SequenceVariant | denotes | p.Asp70Gly |
T10 | 1891-1902 | SequenceVariant | denotes | p.Ala539Thr |
LitCoin-GeneOrGeneProduct-v2
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 22-27 | GeneOrGeneProduct | denotes | novel |
T2 | 28-32 | GeneOrGeneProduct | denotes | BCHE |
T3 | 143-164 | GeneOrGeneProduct | denotes | Butyrylcholinesterase |
T4 | 229-235 | GeneOrGeneProduct | denotes | muscle |
T5 | 314-318 | GeneOrGeneProduct | denotes | BCHE |
T6 | 361-374 | GeneOrGeneProduct | denotes | neuromuscular |
T7 | 449-454 | GeneOrGeneProduct | denotes | novel |
T8 | 455-459 | GeneOrGeneProduct | denotes | BCHE |
T9 | 614-623 | GeneOrGeneProduct | denotes | catalytic |
T10 | 698-719 | GeneOrGeneProduct | denotes | butyrylcholinesterase |
T11 | 837-845 | GeneOrGeneProduct | denotes | atypical |
T12 | 959-966 | GeneOrGeneProduct | denotes | reduced |
T13 | 988-994 | GeneOrGeneProduct | denotes | enzyme |
T14 | 1014-1019 | GeneOrGeneProduct | denotes | minor |
T15 | 1020-1024 | GeneOrGeneProduct | denotes | fast |
T16 | 1077-1084 | GeneOrGeneProduct | denotes | albumin |
T17 | 1099-1106 | GeneOrGeneProduct | denotes | missing |
T18 | 1208-1216 | GeneOrGeneProduct | denotes | behavior |
T19 | 1249-1258 | GeneOrGeneProduct | denotes | catalytic |
T20 | 1325-1333 | GeneOrGeneProduct | denotes | atypical |
T21 | 1334-1340 | GeneOrGeneProduct | denotes | enzyme |
T22 | 1628-1633 | GeneOrGeneProduct | denotes | other |
T23 | 1668-1677 | GeneOrGeneProduct | denotes | catalytic |
T24 | 1759-1765 | GeneOrGeneProduct | denotes | enzyme |
T25 | 1843-1850 | GeneOrGeneProduct | denotes | reduced |
T26 | 1914-1920 | GeneOrGeneProduct | denotes | enzyme |
T27 | 1954-1958 | GeneOrGeneProduct | denotes | fast |
LitCoin-GeneOrGeneProduct-v0
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 22-27 | GeneOrGeneProduct | denotes | novel |
T2 | 28-32 | GeneOrGeneProduct | denotes | BCHE |
T3 | 50-55 | GeneOrGeneProduct | denotes | point |
T4 | 56-64 | GeneOrGeneProduct | denotes | mutation |
T5 | 94-102 | GeneOrGeneProduct | denotes | activity |
T6 | 143-164 | GeneOrGeneProduct | denotes | Butyrylcholinesterase |
T7 | 229-235 | GeneOrGeneProduct | denotes | muscle |
T8 | 236-245 | GeneOrGeneProduct | denotes | relaxants |
T9 | 297-306 | GeneOrGeneProduct | denotes | mutations |
T10 | 314-318 | GeneOrGeneProduct | denotes | BCHE |
T11 | 343-347 | GeneOrGeneProduct | denotes | case |
T12 | 361-374 | GeneOrGeneProduct | denotes | neuromuscular |
T13 | 375-380 | GeneOrGeneProduct | denotes | block |
T14 | 449-454 | GeneOrGeneProduct | denotes | novel |
T15 | 455-459 | GeneOrGeneProduct | denotes | BCHE |
T16 | 592-600 | GeneOrGeneProduct | denotes | mutation |
T17 | 601-609 | GeneOrGeneProduct | denotes | disrupts |
T18 | 614-623 | GeneOrGeneProduct | denotes | catalytic |
T19 | 634-644 | GeneOrGeneProduct | denotes | determines |
T20 | 671-679 | GeneOrGeneProduct | denotes | activity |
T21 | 698-719 | GeneOrGeneProduct | denotes | butyrylcholinesterase |
T22 | 774-780 | GeneOrGeneProduct | denotes | values |
T23 | 837-845 | GeneOrGeneProduct | denotes | atypical |
T24 | 898-902 | GeneOrGeneProduct | denotes | BChE |
T25 | 953-958 | GeneOrGeneProduct | denotes | has a |
T26 | 959-966 | GeneOrGeneProduct | denotes | reduced |
T27 | 988-994 | GeneOrGeneProduct | denotes | enzyme |
T28 | 1014-1019 | GeneOrGeneProduct | denotes | minor |
T29 | 1020-1024 | GeneOrGeneProduct | denotes | fast |
T30 | 1032-1036 | GeneOrGeneProduct | denotes | BChE |
T31 | 1077-1084 | GeneOrGeneProduct | denotes | albumin |
T32 | 1099-1106 | GeneOrGeneProduct | denotes | missing |
T33 | 1176-1180 | GeneOrGeneProduct | denotes | BChE |
T34 | 1192-1196 | GeneOrGeneProduct | denotes | pure |
T35 | 1208-1216 | GeneOrGeneProduct | denotes | behavior |
T36 | 1249-1258 | GeneOrGeneProduct | denotes | catalytic |
T37 | 1295-1300 | GeneOrGeneProduct | denotes | times |
T38 | 1325-1333 | GeneOrGeneProduct | denotes | atypical |
T39 | 1334-1340 | GeneOrGeneProduct | denotes | enzyme |
T40 | 1392-1400 | GeneOrGeneProduct | denotes | activity |
T41 | 1497-1505 | GeneOrGeneProduct | denotes | mutation |
T42 | 1521-1531 | GeneOrGeneProduct | denotes | disruption |
T43 | 1544-1551 | GeneOrGeneProduct | denotes | bonding |
T44 | 1628-1633 | GeneOrGeneProduct | denotes | other |
T45 | 1650-1660 | GeneOrGeneProduct | denotes | disruption |
T46 | 1668-1677 | GeneOrGeneProduct | denotes | catalytic |
T47 | 1759-1765 | GeneOrGeneProduct | denotes | enzyme |
T48 | 1843-1850 | GeneOrGeneProduct | denotes | reduced |
T49 | 1914-1920 | GeneOrGeneProduct | denotes | enzyme |
T50 | 1954-1958 | GeneOrGeneProduct | denotes | fast |
T51 | 1966-1971 | GeneOrGeneProduct | denotes | bands |
T52 | 1991-1995 | GeneOrGeneProduct | denotes | gels |
LitCoin-Disease-MeSH
Id | Subject | Object | Predicate | Lexical cue | originalLabel |
---|---|---|---|---|---|
T1 | 117-122 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 |
T2 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | C537417 |
T3 | 206-211 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 |
LitCoin-GeneOrGeneProduct-v3
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 28-32 | GeneOrGeneProduct | denotes | BCHE |
T2 | 143-164 | GeneOrGeneProduct | denotes | Butyrylcholinesterase |
T3 | 314-318 | GeneOrGeneProduct | denotes | BCHE |
T4 | 361-374 | GeneOrGeneProduct | denotes | neuromuscular |
T5 | 455-459 | GeneOrGeneProduct | denotes | BCHE |
T6 | 698-719 | GeneOrGeneProduct | denotes | butyrylcholinesterase |
T7 | 1077-1084 | GeneOrGeneProduct | denotes | albumin |
T8 | 1099-1106 | GeneOrGeneProduct | denotes | missing |
LitCoin_Mondo_095
Id | Subject | Object | Predicate | Lexical cue | mondo_id |
---|---|---|---|---|---|
T1 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | 0015270 |
T2 | 1443-1445 | DiseaseOrPhenotypicFeature | denotes | MD | 0010651 |
T3 | 1735-1737 | DiseaseOrPhenotypicFeature | denotes | MD | 0010651 |
LitCoin-MeSH-Disease-2
Id | Subject | Object | Predicate | Lexical cue | ID: |
---|---|---|---|---|---|
T1 | 117-122 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 |
T2 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | C537417 |
T3 | 206-211 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 |
T4 | 361-380 | DiseaseOrPhenotypicFeature | denotes | neuromuscular block | DISEASE |
LitCoin-MONDO_bioort2019
Id | Subject | Object | Predicate | Lexical cue | #label |
---|---|---|---|---|---|
T1 | 117-122 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 |
T2 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | C537417 |
T3 | 206-211 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 |
T4 | 361-380 | DiseaseOrPhenotypicFeature | denotes | neuromuscular block | DISEASE |
LitCoin-Chemical-MeSH-CHEBI
Id | Subject | Object | Predicate | Lexical cue | ID: |
---|---|---|---|---|---|
T1 | 128-141 | ChemicalEntity | denotes | suxamethonium | D013390|http://purl.obolibrary.org/obo/CHEBI_45652 |
T3 | 143-175 | ChemicalEntity | denotes | Butyrylcholinesterase deficiency | C537417 |
T4 | 229-245 | ChemicalEntity | denotes | muscle relaxants | ChemicalEntity |
T5 | 247-260 | ChemicalEntity | denotes | suxamethonium | D013390|http://purl.obolibrary.org/obo/CHEBI_45652 |
T7 | 264-274 | ChemicalEntity | denotes | mivacurium | D000077590 |
T8 | 405-418 | ChemicalEntity | denotes | suxamethonium | D013390|http://purl.obolibrary.org/obo/CHEBI_45652 |
T10 | 698-719 | ChemicalEntity | denotes | butyrylcholinesterase | D002091 |
T11 | 725-743 | ChemicalEntity | denotes | butyrylthiocholine | D002092|http://purl.obolibrary.org/obo/CHEBI_41055 |
T13 | 745-748 | ChemicalEntity | denotes | BTC | D002092 |
T14 | 754-768 | ChemicalEntity | denotes | benzoylcholine | D001588|ChemicalEntity |
T16 | 784-793 | ChemicalEntity | denotes | dibucaine | D003992|http://purl.obolibrary.org/obo/CHEBI_247956 |
T18 | 798-806 | ChemicalEntity | denotes | fluoride | D005459|http://purl.obolibrary.org/obo/CHEBI_17051 |
T20 | 1222-1225 | ChemicalEntity | denotes | BTC | D002092 |
T21 | 1286-1289 | ChemicalEntity | denotes | BTC | D002092 |
T22 | 1409-1422 | ChemicalEntity | denotes | suxamethonium | D013390|http://purl.obolibrary.org/obo/CHEBI_45652 |
T24 | 1535-1543 | ChemicalEntity | denotes | hydrogen | D006859|http://purl.obolibrary.org/obo/CHEBI_49637 |
T26 | 1991-1995 | ChemicalEntity | denotes | gels | D005782 |
LitCoin-NCBITaxon-2
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 279-287 | OrganismTaxon | denotes | patients |
T2 | 683-690 | OrganismTaxon | denotes | patient |
T3 | 945-952 | OrganismTaxon | denotes | patient |
LitCoin-training-merged
Id | Subject | Object | Predicate | Lexical cue | #label | ID: |
---|---|---|---|---|---|---|
T26 | 1991-1995 | ChemicalEntity | denotes | gels | D005782 | |
T24 | 1535-1543 | ChemicalEntity | denotes | hydrogen | http://purl.obolibrary.org/obo/CHEBI_49637|D006859 | |
T22 | 1409-1422 | ChemicalEntity | denotes | suxamethonium | http://purl.obolibrary.org/obo/CHEBI_45652|D013390 | |
T21 | 1286-1289 | ChemicalEntity | denotes | BTC | D002092 | |
T20 | 1222-1225 | ChemicalEntity | denotes | BTC | D002092 | |
T18 | 798-806 | ChemicalEntity | denotes | fluoride | http://purl.obolibrary.org/obo/CHEBI_17051|D005459 | |
T16 | 784-793 | ChemicalEntity | denotes | dibucaine | http://purl.obolibrary.org/obo/CHEBI_247956|D003992 | |
T14 | 754-768 | ChemicalEntity | denotes | benzoylcholine | ChemicalEntity|D001588 | |
T13 | 745-748 | ChemicalEntity | denotes | BTC | D002092 | |
T11 | 725-743 | ChemicalEntity | denotes | butyrylthiocholine | http://purl.obolibrary.org/obo/CHEBI_41055|D002092 | |
T10 | 698-719 | ChemicalEntity | denotes | butyrylcholinesterase | D002091 | |
T8 | 405-418 | ChemicalEntity | denotes | suxamethonium | http://purl.obolibrary.org/obo/CHEBI_45652|D013390 | |
T7 | 264-274 | ChemicalEntity | denotes | mivacurium | D000077590 | |
T5 | 247-260 | ChemicalEntity | denotes | suxamethonium | http://purl.obolibrary.org/obo/CHEBI_45652|D013390 | |
T4 | 229-245 | ChemicalEntity | denotes | muscle relaxants | ChemicalEntity | |
T3 | 143-175 | ChemicalEntity | denotes | Butyrylcholinesterase deficiency | C537417 | |
T1 | 128-141 | ChemicalEntity | denotes | suxamethonium | http://purl.obolibrary.org/obo/CHEBI_45652|D013390 | |
T49719 | 1099-1106 | GeneOrGeneProduct | denotes | missing | ||
T49043 | 1077-1084 | GeneOrGeneProduct | denotes | albumin | ||
T6 | 698-719 | GeneOrGeneProduct | denotes | butyrylcholinesterase | ||
T26166 | 455-459 | GeneOrGeneProduct | denotes | BCHE | ||
T58069 | 361-374 | GeneOrGeneProduct | denotes | neuromuscular | ||
T53614 | 314-318 | GeneOrGeneProduct | denotes | BCHE | ||
T2 | 143-164 | GeneOrGeneProduct | denotes | Butyrylcholinesterase | ||
T44130 | 28-32 | GeneOrGeneProduct | denotes | BCHE | ||
T79380 | 361-380 | DiseaseOrPhenotypicFeature | denotes | neuromuscular block | DISEASE | |
T85129 | 206-211 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 | |
T57186 | 143-175 | DiseaseOrPhenotypicFeature | denotes | Butyrylcholinesterase deficiency | C537417 | |
T88658 | 117-122 | DiseaseOrPhenotypicFeature | denotes | apnea | D001049 | |
T31189 | 945-952 | OrganismTaxon | denotes | patient | ||
T4769 | 683-690 | OrganismTaxon | denotes | patient | ||
T32204 | 279-287 | OrganismTaxon | denotes | patients | ||
T87511 | 1891-1902 | SequenceVariant | denotes | p.Ala539Thr | ||
T9 | 1880-1890 | SequenceVariant | denotes | p.Asp70Gly | ||
T98533 | 1868-1879 | SequenceVariant | denotes | p.Ala204Asp | ||
T91485 | 1506-1517 | SequenceVariant | denotes | p.Val204Asp | ||
T60846 | 1164-1175 | SequenceVariant | denotes | p.Ala539Thr | ||
T21164 | 1153-1163 | SequenceVariant | denotes | p.Asp70Gly | ||
T56352 | 1141-1152 | SequenceVariant | denotes | p.Val204Asp | ||
T42118 | 479-490 | SequenceVariant | denotes | p.Val204Asp | ||
T28157 | 469-477 | SequenceVariant | denotes | c.695T>A | ||
T10402 | 66-77 | SequenceVariant | denotes | p.Val204Asp |
sentences
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
TextSentencer_T1 | 0-142 | Sentence | denotes | Characterization of a novel BCHE "silent" allele: point mutation (p.Val204Asp) causes loss of activity and prolonged apnea with suxamethonium. |
TextSentencer_T2 | 143-324 | Sentence | denotes | Butyrylcholinesterase deficiency is characterized by prolonged apnea after the use of muscle relaxants (suxamethonium or mivacurium) in patients who have mutations in the BCHE gene. |
TextSentencer_T3 | 325-492 | Sentence | denotes | Here, we report a case of prolonged neuromuscular block after administration of suxamethonium leading to the discovery of a novel BCHE variant (c.695T>A, p.Val204Asp). |
TextSentencer_T4 | 493-666 | Sentence | denotes | Inhibition studies, kinetic analysis and molecular dynamics were undertaken to understand how this mutation disrupts the catalytic triad and determines a "silent" phenotype. |
TextSentencer_T5 | 667-862 | Sentence | denotes | Low activity of patient plasma butyrylcholinesterase with butyrylthiocholine (BTC) and benzoylcholine, and values of dibucaine and fluoride numbers fit with heterozygous atypical silent genotype. |
TextSentencer_T6 | 863-1107 | Sentence | denotes | Electrophoretic analysis of plasma BChE of the proband and his mother showed that patient has a reduced amount of tetrameric enzyme in plasma and that minor fast-moving BChE components: monomer, dimer, and monomer-albumin conjugate are missing. |
TextSentencer_T7 | 1108-1243 | Sentence | denotes | Kinetic analysis showed that the p.Val204Asp/p.Asp70Gly-p.Ala539Thr BChE displays a pure Michaelian behavior with BTC as the substrate. |
TextSentencer_T8 | 1244-1423 | Sentence | denotes | Both catalytic parameters Km = 265 µM for BTC, two times higher than that of the atypical enzyme, and a low Vmax are consistent with the absence of activity against suxamethonium. |
TextSentencer_T9 | 1424-1734 | Sentence | denotes | Molecular dynamic (MD) simulations showed that the overall effect of the mutation p.Val204Asp is disruption of hydrogen bonding between Gln223 and Glu441, leading Ser198 and His438 to move away from each other with subsequent disruption of the catalytic triad functionality regardless of the type of substrate. |
TextSentencer_T10 | 1735-1823 | Sentence | denotes | MD also showed that the enzyme volume is increased, suggesting a pre-denaturation state. |
TextSentencer_T11 | 1824-1996 | Sentence | denotes | This fits with the reduced concentration of p.Ala204Asp/p.Asp70Gly-p.Ala539Thr tetrameric enzyme in the plasma and non-detectable fast moving-bands on electrophoresis gels. |
T1 | 0-142 | Sentence | denotes | Characterization of a novel BCHE "silent" allele: point mutation (p.Val204Asp) causes loss of activity and prolonged apnea with suxamethonium. |
T2 | 143-324 | Sentence | denotes | Butyrylcholinesterase deficiency is characterized by prolonged apnea after the use of muscle relaxants (suxamethonium or mivacurium) in patients who have mutations in the BCHE gene. |
T3 | 325-492 | Sentence | denotes | Here, we report a case of prolonged neuromuscular block after administration of suxamethonium leading to the discovery of a novel BCHE variant (c.695T>A, p.Val204Asp). |
T4 | 493-666 | Sentence | denotes | Inhibition studies, kinetic analysis and molecular dynamics were undertaken to understand how this mutation disrupts the catalytic triad and determines a "silent" phenotype. |
T5 | 667-862 | Sentence | denotes | Low activity of patient plasma butyrylcholinesterase with butyrylthiocholine (BTC) and benzoylcholine, and values of dibucaine and fluoride numbers fit with heterozygous atypical silent genotype. |
T6 | 863-1107 | Sentence | denotes | Electrophoretic analysis of plasma BChE of the proband and his mother showed that patient has a reduced amount of tetrameric enzyme in plasma and that minor fast-moving BChE components: monomer, dimer, and monomer-albumin conjugate are missing. |
T7 | 1108-1243 | Sentence | denotes | Kinetic analysis showed that the p.Val204Asp/p.Asp70Gly-p.Ala539Thr BChE displays a pure Michaelian behavior with BTC as the substrate. |
T8 | 1244-1423 | Sentence | denotes | Both catalytic parameters Km = 265 µM for BTC, two times higher than that of the atypical enzyme, and a low Vmax are consistent with the absence of activity against suxamethonium. |
T9 | 1424-1734 | Sentence | denotes | Molecular dynamic (MD) simulations showed that the overall effect of the mutation p.Val204Asp is disruption of hydrogen bonding between Gln223 and Glu441, leading Ser198 and His438 to move away from each other with subsequent disruption of the catalytic triad functionality regardless of the type of substrate. |
T10 | 1735-1823 | Sentence | denotes | MD also showed that the enzyme volume is increased, suggesting a pre-denaturation state. |
T11 | 1824-1996 | Sentence | denotes | This fits with the reduced concentration of p.Ala204Asp/p.Asp70Gly-p.Ala539Thr tetrameric enzyme in the plasma and non-detectable fast moving-bands on electrophoresis gels. |
PubmedHPO
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 206-211 | HP_0002104 | denotes | apnea |
Allie
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
SS1_25054547_4_0 | 725-743 | expanded | denotes | butyrylthiocholine |
SS2_25054547_4_0 | 745-748 | abbr | denotes | BTC |
SS1_25054547_8_0 | 1424-1441 | expanded | denotes | Molecular dynamic |
SS2_25054547_8_0 | 1443-1445 | abbr | denotes | MD |
AE1_25054547_4_0 | SS1_25054547_4_0 | SS2_25054547_4_0 | abbreviatedTo | butyrylthiocholine,BTC |
AE1_25054547_8_0 | SS1_25054547_8_0 | SS2_25054547_8_0 | abbreviatedTo | Molecular dynamic,MD |
DisGeNET5_gene_disease
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
25054547-2#130#134#gene590 | 455-459 | gene590 | denotes | BCHE |
25054547-2#26#55#diseaseC0520758 | 351-380 | diseaseC0520758 | denotes | prolonged neuromuscular block |
130#134#gene59026#55#diseaseC0520758 | 25054547-2#130#134#gene590 | 25054547-2#26#55#diseaseC0520758 | associated_with | BCHE,prolonged neuromuscular block |
PubCasesHPO
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
AB1 | 206-211 | HP:0002104 | denotes | apnea |
TI1 | 117-122 | HP:0002104 | denotes | apnea |
PubCasesORDO
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
AB1 | 143-175 | ORDO:132 | denotes | Butyrylcholinesterase deficiency |