PubMed:19394258
Annnotations
LitCoin-sentences
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 0-101 | Sentence | denotes | The first founder DGUOK mutation associated with hepatocerebral mitochondrial DNA depletion syndrome. |
T2 | 102-235 | Sentence | denotes | Deoxyguanosine kinase (dGK) deficiency is a frequent cause of mitochondrial DNA depletion associated with a hepatocerebral phenotype. |
T3 | 236-383 | Sentence | denotes | In this study, we describe a new splice site mutation in the DGUOK gene and the clinical, radiologic, and genetic features of these DGUOK patients. |
T4 | 384-603 | Sentence | denotes | This new DGUOK homozygous mutation (c.444-62C>A) was identified in three patients from two North-African consanguineous families with combined respiratory chain deficiencies and mitochondrial DNA depletion in the liver. |
T5 | 604-662 | Sentence | denotes | Brain MRIs are normal in DGUOK patients in the literature. |
T6 | 663-790 | Sentence | denotes | Interestingly, we found subtentorial abnormal myelination and moderate hyperintensity in the bilateral pallidi in our patients. |
T7 | 791-961 | Sentence | denotes | This new mutation creates a cryptic splice site in intron 3 (in position -62) and is predicted to result in a larger protein with an in-frame insertion of 20 amino acids. |
T8 | 962-1200 | Sentence | denotes | In silico analysis of the putative impact of the insertion shows serious clashes in protein conformation: this insertion disrupts the alpha5 helix of the dGK kinase domain, rendering the protein unable to bind purine deoxyribonucleosides. |
T9 | 1201-1418 | Sentence | denotes | In addition, a common haplotype that segregated with the disease in both families was detected by haplotype reconstruction with 10 markers (microsatellites and SNPs), which span 4.6 Mb of DNA covering the DGUOK locus. |
T10 | 1419-1610 | Sentence | denotes | In conclusion, we report a new DGUOK splice site mutation that provide insight into a critical protein domain (dGK kinase domain) and the first founder mutation in a North-African population. |
LitCoin-entities
Id | Subject | Object | Predicate | Lexical cue | db_id |
---|---|---|---|---|---|
6019 | 18-23 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6020 | 49-100 | DiseaseOrPhenotypicFeature | denotes | hepatocerebral mitochondrial DNA depletion syndrome | MESH:C580039 |
6021 | 102-140 | DiseaseOrPhenotypicFeature | denotes | Deoxyguanosine kinase (dGK) deficiency | MESH:C580039 |
6022 | 164-191 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | MESH:C580039 |
6023 | 297-302 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6024 | 368-373 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6025 | 374-382 | OrganismTaxon | denotes | patients | NCBITaxon:9606 |
6026 | 393-398 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6027 | 420-431 | SequenceVariant | denotes | c.444-62C>A | c|SUB|C|444-62|A |
6028 | 457-465 | OrganismTaxon | denotes | patients | NCBITaxon:9606 |
6029 | 527-557 | DiseaseOrPhenotypicFeature | denotes | respiratory chain deficiencies | MESH:D028361 |
6030 | 562-589 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | MESH:C580039 |
6031 | 629-634 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6032 | 635-643 | OrganismTaxon | denotes | patients | NCBITaxon:9606 |
6033 | 687-720 | DiseaseOrPhenotypicFeature | denotes | subtentorial abnormal myelination | |
6034 | 725-748 | DiseaseOrPhenotypicFeature | denotes | moderate hyperintensity | MESH:D006973 |
6035 | 781-789 | OrganismTaxon | denotes | patients | NCBITaxon:9606 |
6036 | 1116-1119 | GeneOrGeneProduct | denotes | dGK | NCBIGene:1716 |
6037 | 1179-1199 | ChemicalEntity | denotes | deoxyribonucleosides | MESH:D003853 |
6038 | 1406-1411 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6039 | 1450-1455 | GeneOrGeneProduct | denotes | DGUOK | NCBIGene:1716 |
6040 | 1530-1533 | GeneOrGeneProduct | denotes | dGK | NCBIGene:1716 |
LitCoin_Mondo
Id | Subject | Object | Predicate | Lexical cue | mondo_id |
---|---|---|---|---|---|
T1 | 64-100 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion syndrome | 0018158 |
LitCoin-SeqVar
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 420-431 | SequenceVariant | denotes | c.444-62C>A |
LitCoin-GeneOrGeneProduct-v0
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 18-23 | GeneOrGeneProduct | denotes | DGUOK |
T2 | 24-32 | GeneOrGeneProduct | denotes | mutation |
T3 | 64-77 | GeneOrGeneProduct | denotes | mitochondrial |
T4 | 82-91 | GeneOrGeneProduct | denotes | depletion |
T5 | 92-100 | GeneOrGeneProduct | denotes | syndrome |
T6 | 102-123 | GeneOrGeneProduct | denotes | Deoxyguanosine kinase |
T7 | 125-128 | GeneOrGeneProduct | denotes | dGK |
T8 | 164-177 | GeneOrGeneProduct | denotes | mitochondrial |
T9 | 182-191 | GeneOrGeneProduct | denotes | depletion |
T10 | 269-275 | GeneOrGeneProduct | denotes | splice |
T11 | 281-289 | GeneOrGeneProduct | denotes | mutation |
T12 | 297-302 | GeneOrGeneProduct | denotes | DGUOK |
T13 | 368-373 | GeneOrGeneProduct | denotes | DGUOK |
T14 | 393-398 | GeneOrGeneProduct | denotes | DGUOK |
T15 | 410-418 | GeneOrGeneProduct | denotes | mutation |
T16 | 539-544 | GeneOrGeneProduct | denotes | chain |
T17 | 562-575 | GeneOrGeneProduct | denotes | mitochondrial |
T18 | 580-589 | GeneOrGeneProduct | denotes | depletion |
T19 | 597-602 | GeneOrGeneProduct | denotes | liver |
T20 | 604-609 | GeneOrGeneProduct | denotes | Brain |
T21 | 629-634 | GeneOrGeneProduct | denotes | DGUOK |
T22 | 800-808 | GeneOrGeneProduct | denotes | mutation |
T23 | 809-816 | GeneOrGeneProduct | denotes | creates |
T24 | 819-826 | GeneOrGeneProduct | denotes | cryptic |
T25 | 827-833 | GeneOrGeneProduct | denotes | splice |
T26 | 876-885 | GeneOrGeneProduct | denotes | predicted |
T27 | 896-900 | GeneOrGeneProduct | denotes | in a |
T28 | 908-915 | GeneOrGeneProduct | denotes | protein |
T29 | 949-960 | GeneOrGeneProduct | denotes | amino acids |
T30 | 988-996 | GeneOrGeneProduct | denotes | putative |
T31 | 997-1003 | GeneOrGeneProduct | denotes | impact |
T32 | 1046-1053 | GeneOrGeneProduct | denotes | protein |
T33 | 1083-1091 | GeneOrGeneProduct | denotes | disrupts |
T34 | 1096-1102 | GeneOrGeneProduct | denotes | alpha5 |
T35 | 1103-1108 | GeneOrGeneProduct | denotes | helix |
T36 | 1116-1119 | GeneOrGeneProduct | denotes | dGK |
T37 | 1120-1126 | GeneOrGeneProduct | denotes | kinase |
T38 | 1149-1156 | GeneOrGeneProduct | denotes | protein |
T39 | 1167-1171 | GeneOrGeneProduct | denotes | bind |
T40 | 1179-1199 | GeneOrGeneProduct | denotes | deoxyribonucleosides |
T41 | 1374-1378 | GeneOrGeneProduct | denotes | span |
T42 | 1383-1385 | GeneOrGeneProduct | denotes | Mb |
T43 | 1406-1411 | GeneOrGeneProduct | denotes | DGUOK |
T44 | 1450-1455 | GeneOrGeneProduct | denotes | DGUOK |
T45 | 1456-1462 | GeneOrGeneProduct | denotes | splice |
T46 | 1468-1481 | GeneOrGeneProduct | denotes | mutation that |
T47 | 1514-1521 | GeneOrGeneProduct | denotes | protein |
T48 | 1530-1533 | GeneOrGeneProduct | denotes | dGK |
T49 | 1534-1540 | GeneOrGeneProduct | denotes | kinase |
T50 | 1571-1579 | GeneOrGeneProduct | denotes | mutation |
LitCoin-GeneOrGeneProduct-v2
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 18-23 | GeneOrGeneProduct | denotes | DGUOK |
T2 | 64-77 | GeneOrGeneProduct | denotes | mitochondrial |
T3 | 92-100 | GeneOrGeneProduct | denotes | syndrome |
T4 | 102-123 | GeneOrGeneProduct | denotes | Deoxyguanosine kinase |
T5 | 125-128 | GeneOrGeneProduct | denotes | dGK |
T6 | 164-177 | GeneOrGeneProduct | denotes | mitochondrial |
T7 | 297-302 | GeneOrGeneProduct | denotes | DGUOK |
T8 | 368-373 | GeneOrGeneProduct | denotes | DGUOK |
T9 | 393-398 | GeneOrGeneProduct | denotes | DGUOK |
T10 | 539-544 | GeneOrGeneProduct | denotes | chain |
T11 | 562-575 | GeneOrGeneProduct | denotes | mitochondrial |
T12 | 597-602 | GeneOrGeneProduct | denotes | liver |
T13 | 604-609 | GeneOrGeneProduct | denotes | Brain |
T14 | 629-634 | GeneOrGeneProduct | denotes | DGUOK |
T15 | 819-826 | GeneOrGeneProduct | denotes | cryptic |
T16 | 908-915 | GeneOrGeneProduct | denotes | protein |
T17 | 949-960 | GeneOrGeneProduct | denotes | amino acids |
T18 | 988-996 | GeneOrGeneProduct | denotes | putative |
T19 | 997-1003 | GeneOrGeneProduct | denotes | impact |
T20 | 1046-1053 | GeneOrGeneProduct | denotes | protein |
T21 | 1096-1102 | GeneOrGeneProduct | denotes | alpha5 |
T22 | 1103-1108 | GeneOrGeneProduct | denotes | helix |
T23 | 1116-1119 | GeneOrGeneProduct | denotes | dGK |
T24 | 1120-1126 | GeneOrGeneProduct | denotes | kinase |
T25 | 1149-1156 | GeneOrGeneProduct | denotes | protein |
T26 | 1179-1199 | GeneOrGeneProduct | denotes | deoxyribonucleosides |
T27 | 1374-1378 | GeneOrGeneProduct | denotes | span |
T28 | 1406-1411 | GeneOrGeneProduct | denotes | DGUOK |
T29 | 1450-1455 | GeneOrGeneProduct | denotes | DGUOK |
T30 | 1514-1521 | GeneOrGeneProduct | denotes | protein |
T31 | 1530-1533 | GeneOrGeneProduct | denotes | dGK |
T32 | 1534-1540 | GeneOrGeneProduct | denotes | kinase |
LitCoin-Disease-MeSH
Id | Subject | Object | Predicate | Lexical cue | originalLabel |
---|---|---|---|---|---|
T1 | 49-100 | DiseaseOrPhenotypicFeature | denotes | hepatocerebral mitochondrial DNA depletion syndrome | C580039 |
T2 | 164-191 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE |
T3 | 527-557 | DiseaseOrPhenotypicFeature | denotes | respiratory chain deficiencies | D028361 |
T4 | 562-589 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE |
T5 | 1258-1265 | DiseaseOrPhenotypicFeature | denotes | disease | D004194 |
LitCoin-GeneOrGeneProduct-v3
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 18-23 | GeneOrGeneProduct | denotes | DGUOK |
T2 | 102-123 | GeneOrGeneProduct | denotes | Deoxyguanosine kinase |
T3 | 125-128 | GeneOrGeneProduct | denotes | dGK |
T4 | 297-302 | GeneOrGeneProduct | denotes | DGUOK |
T5 | 368-373 | GeneOrGeneProduct | denotes | DGUOK |
T6 | 393-398 | GeneOrGeneProduct | denotes | DGUOK |
T7 | 629-634 | GeneOrGeneProduct | denotes | DGUOK |
T8 | 988-1003 | GeneOrGeneProduct | denotes | putative impact |
T9 | 1096-1102 | GeneOrGeneProduct | denotes | alpha5 |
T10 | 1116-1119 | GeneOrGeneProduct | denotes | dGK |
T11 | 1179-1199 | GeneOrGeneProduct | denotes | deoxyribonucleosides |
T12 | 1374-1378 | GeneOrGeneProduct | denotes | span |
T13 | 1406-1411 | GeneOrGeneProduct | denotes | DGUOK |
T14 | 1450-1455 | GeneOrGeneProduct | denotes | DGUOK |
T15 | 1530-1533 | GeneOrGeneProduct | denotes | dGK |
LitCoin_Mondo_095
Id | Subject | Object | Predicate | Lexical cue | mondo_id |
---|---|---|---|---|---|
T1 | 64-100 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion syndrome | 0018158 |
LitCoin-MeSH-Disease-2
Id | Subject | Object | Predicate | Lexical cue | ID: |
---|---|---|---|---|---|
T1 | 49-100 | DiseaseOrPhenotypicFeature | denotes | hepatocerebral mitochondrial DNA depletion syndrome | C580039 |
T2 | 102-140 | DiseaseOrPhenotypicFeature | denotes | Deoxyguanosine kinase (dGK) deficiency | DISEASE |
T3 | 164-191 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE |
T4 | 527-557 | DiseaseOrPhenotypicFeature | denotes | respiratory chain deficiencies | D028361 |
T5 | 562-589 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE |
T6 | 687-720 | DiseaseOrPhenotypicFeature | denotes | subtentorial abnormal myelination | DISEASE |
T7 | 725-748 | DiseaseOrPhenotypicFeature | denotes | moderate hyperintensity | DISEASE |
T8 | 1258-1265 | DiseaseOrPhenotypicFeature | denotes | disease | D004194 |
LitCoin-MONDO_bioort2019
Id | Subject | Object | Predicate | Lexical cue | #label |
---|---|---|---|---|---|
T1 | 49-100 | DiseaseOrPhenotypicFeature | denotes | hepatocerebral mitochondrial DNA depletion syndrome | C580039 |
T2 | 102-140 | DiseaseOrPhenotypicFeature | denotes | Deoxyguanosine kinase (dGK) deficiency | DISEASE |
T3 | 164-191 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE |
T4 | 527-557 | DiseaseOrPhenotypicFeature | denotes | respiratory chain deficiencies | D028361 |
T5 | 562-589 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE |
T6 | 687-720 | DiseaseOrPhenotypicFeature | denotes | subtentorial abnormal myelination | DISEASE |
T7 | 725-748 | DiseaseOrPhenotypicFeature | denotes | moderate hyperintensity | DISEASE |
LitCoin-NCBITaxon-2
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 374-382 | OrganismTaxon | denotes | patients |
T2 | 457-465 | OrganismTaxon | denotes | patients |
T3 | 635-643 | OrganismTaxon | denotes | patients |
T4 | 781-789 | OrganismTaxon | denotes | patients |
LitCoin-Chemical-MeSH-CHEBI
Id | Subject | Object | Predicate | Lexical cue | ID: |
---|---|---|---|---|---|
T1 | 102-123 | ChemicalEntity | denotes | Deoxyguanosine kinase | C019165 |
T2 | 125-128 | ChemicalEntity | denotes | dGK | C019165 |
T3 | 1116-1119 | ChemicalEntity | denotes | dGK | C019165 |
T4 | 1172-1199 | ChemicalEntity | denotes | purine deoxyribonucleosides | http://purl.obolibrary.org/obo/CHEBI_60173 |
T5 | 1530-1533 | ChemicalEntity | denotes | dGK | C019165 |
LitCoin-training-merged
Id | Subject | Object | Predicate | Lexical cue | #label | ID: |
---|---|---|---|---|---|---|
T5 | 1530-1533 | ChemicalEntity | denotes | dGK | C019165 | |
T4 | 1172-1199 | ChemicalEntity | denotes | purine deoxyribonucleosides | http://purl.obolibrary.org/obo/CHEBI_60173 | |
T3 | 1116-1119 | ChemicalEntity | denotes | dGK | C019165 | |
T2 | 125-128 | ChemicalEntity | denotes | dGK | C019165 | |
T1 | 102-123 | ChemicalEntity | denotes | Deoxyguanosine kinase | C019165 | |
T15 | 1530-1533 | GeneOrGeneProduct | denotes | dGK | ||
T14 | 1450-1455 | GeneOrGeneProduct | denotes | DGUOK | ||
T13 | 1406-1411 | GeneOrGeneProduct | denotes | DGUOK | ||
T12 | 1374-1378 | GeneOrGeneProduct | denotes | span | ||
T11 | 1179-1199 | GeneOrGeneProduct | denotes | deoxyribonucleosides | ||
T10 | 1116-1119 | GeneOrGeneProduct | denotes | dGK | ||
T9 | 1096-1102 | GeneOrGeneProduct | denotes | alpha5 | ||
T8 | 988-1003 | GeneOrGeneProduct | denotes | putative impact | ||
T7 | 629-634 | GeneOrGeneProduct | denotes | DGUOK | ||
T6 | 393-398 | GeneOrGeneProduct | denotes | DGUOK | ||
T88655 | 368-373 | GeneOrGeneProduct | denotes | DGUOK | ||
T42015 | 297-302 | GeneOrGeneProduct | denotes | DGUOK | ||
T12124 | 125-128 | GeneOrGeneProduct | denotes | dGK | ||
T24709 | 102-123 | GeneOrGeneProduct | denotes | Deoxyguanosine kinase | ||
T93033 | 18-23 | GeneOrGeneProduct | denotes | DGUOK | ||
T23871 | 725-748 | DiseaseOrPhenotypicFeature | denotes | moderate hyperintensity | DISEASE | |
T90268 | 687-720 | DiseaseOrPhenotypicFeature | denotes | subtentorial abnormal myelination | DISEASE | |
T99729 | 562-589 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE | |
T1799 | 527-557 | DiseaseOrPhenotypicFeature | denotes | respiratory chain deficiencies | D028361 | |
T71838 | 164-191 | DiseaseOrPhenotypicFeature | denotes | mitochondrial DNA depletion | DISEASE | |
T51318 | 102-140 | DiseaseOrPhenotypicFeature | denotes | Deoxyguanosine kinase (dGK) deficiency | DISEASE | |
T91302 | 49-100 | DiseaseOrPhenotypicFeature | denotes | hepatocerebral mitochondrial DNA depletion syndrome | C580039 | |
T75256 | 781-789 | OrganismTaxon | denotes | patients | ||
T30636 | 635-643 | OrganismTaxon | denotes | patients | ||
T73141 | 457-465 | OrganismTaxon | denotes | patients | ||
T23475 | 374-382 | OrganismTaxon | denotes | patients | ||
T3536 | 420-431 | SequenceVariant | denotes | c.444-62C>A |
DisGeNET
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T0 | 18-23 | gene:1716 | denotes | DGUOK |
T1 | 49-100 | disease:C3711385 | denotes | hepatocerebral mitochondrial DNA depletion syndrome |
T2 | 393-398 | gene:1716 | denotes | DGUOK |
T3 | 562-589 | disease:C0342782 | denotes | mitochondrial DNA depletion |
T4 | 393-398 | gene:1716 | denotes | DGUOK |
T5 | 527-557 | disease:C0949857 | denotes | respiratory chain deficiencies |
R1 | T0 | T1 | associated_with | DGUOK,hepatocerebral mitochondrial DNA depletion syndrome |
R2 | T2 | T3 | associated_with | DGUOK,mitochondrial DNA depletion |
R3 | T4 | T5 | associated_with | DGUOK,respiratory chain deficiencies |
DisGeNET5_gene_disease
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
19394258-0#18#23#gene1716 | 18-23 | gene1716 | denotes | DGUOK |
19394258-0#49#100#diseaseC3711385 | 49-100 | diseaseC3711385 | denotes | hepatocerebral mitochondrial DNA depletion syndrome |
19394258-3#9#14#gene1716 | 393-398 | gene1716 | denotes | DGUOK |
19394258-3#143#173#diseaseC0949857 | 527-557 | diseaseC0949857 | denotes | respiratory chain deficiencies |
19394258-3#178#205#diseaseC0342782 | 562-589 | diseaseC0342782 | denotes | mitochondrial DNA depletion |
18#23#gene171649#100#diseaseC3711385 | 19394258-0#18#23#gene1716 | 19394258-0#49#100#diseaseC3711385 | associated_with | DGUOK,hepatocerebral mitochondrial DNA depletion syndrome |
9#14#gene1716143#173#diseaseC0949857 | 19394258-3#9#14#gene1716 | 19394258-3#143#173#diseaseC0949857 | associated_with | DGUOK,respiratory chain deficiencies |
9#14#gene1716178#205#diseaseC0342782 | 19394258-3#9#14#gene1716 | 19394258-3#178#205#diseaseC0342782 | associated_with | DGUOK,mitochondrial DNA depletion |
PubCasesHPO
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
AB1 | 700-720 | HP:0012447 | denotes | abnormal myelination |
tmVarCorpus
Id | Subject | Object | Predicate | Lexical cue |
---|---|---|---|---|
T1 | 420-431 | DNAMutation:c|SUB|C|444-62|A | denotes | c.444-62C>A |