PubMed:19697366
Annnotations
PubMed_ArguminSci
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| T1 | 186-410 | DRI_Outcome | denotes | The aims of this study were to (1) characterize the clinical phenotype, (2) define the causative mutation, and (3) correlate the clinical phenotype with genotype in a large consanguineous Arab family with myotonia congenita. |
| T2 | 411-491 | DRI_Approach | denotes | Twenty-four family members from three generations were interviewed and examined. |
| T3 | 492-591 | DRI_Background | denotes | Genomic DNA was extracted from peripheral blood samples for sequencing the exons of the CLCN1 gene. |
| T4 | 592-720 | DRI_Approach | denotes | Twelve individuals with myotonia congenita transmitted the condition in an autosomal dominant manner with incomplete penetrance. |
| T5 | 721-815 | DRI_Background | denotes | A novel missense mutation [568GG>TC (G190S)] was found in a dose-dependent clinical phenotype. |
| T6 | 816-938 | DRI_Background | denotes | Although heterozygous individuals were asymptomatic or mildly affected, the homozygous individuals were severely affected. |
| T7 | 939-1098 | DRI_Approach | denotes | The mutation is a glycine-to-serine residue substitution in a well-conserved motif in helix D of the CLC-1 chloride channel in the skeletal muscle plasmalemma. |
| T8 | 1099-1246 | DRI_Background | denotes | A novel mutation, 568GG>TC (G190S) in the CLCN1 gene, is responsible for autosomal dominant myotonia congenita with a variable phenotypic spectrum. |
DisGeNET
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| T0 | 174-179 | gene:1180 | denotes | CLCN1 |
| T1 | 0-18 | disease:C0027127 | denotes | Myotonia congenita |
| T2 | 174-179 | gene:1180 | denotes | CLCN1 |
| T3 | 0-18 | disease:C2936781 | denotes | Myotonia congenita |
| T4 | 1141-1146 | gene:1180 | denotes | CLCN1 |
| T5 | 1172-1209 | disease:C2936781 | denotes | autosomal dominant myotonia congenita |
| R1 | T0 | T1 | associated_with | CLCN1,Myotonia congenita |
| R2 | T2 | T3 | associated_with | CLCN1,Myotonia congenita |
| R3 | T4 | T5 | associated_with | CLCN1,autosomal dominant myotonia congenita |
DisGeNET5_variant_disease
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| 19697366-8#28#33#geners797045032 | 1127-1132 | geners797045032 | denotes | G190S |
| 19697366-8#73#110#diseaseC2936781 | 1172-1209 | diseaseC2936781 | denotes | autosomal dominant myotonia congenita |
| 28#33#geners79704503273#110#diseaseC2936781 | 19697366-8#28#33#geners797045032 | 19697366-8#73#110#diseaseC2936781 | associated_with | G190S,autosomal dominant myotonia congenita |
DisGeNET5_gene_disease
| Id | Subject | Object | Predicate | Lexical cue |
|---|---|---|---|---|
| 19697366-0#174#179#gene1180 | 174-179 | gene1180 | denotes | CLCN1 |
| 19697366-0#0#18#diseaseC0027127 | 0-18 | diseaseC0027127 | denotes | Myotonia congenita |
| 19697366-0#0#18#diseaseC2936781 | 0-18 | diseaseC2936781 | denotes | Myotonia congenita |
| 174#179#gene11800#18#diseaseC0027127 | 19697366-0#174#179#gene1180 | 19697366-0#0#18#diseaseC0027127 | associated_with | CLCN1,Myotonia congenita |
| 174#179#gene11800#18#diseaseC2936781 | 19697366-0#174#179#gene1180 | 19697366-0#0#18#diseaseC2936781 | associated_with | CLCN1,Myotonia congenita |