| Id |
Subject |
Object |
Predicate |
Lexical cue |
| TextSentencer_T1 |
0-86 |
Sentence |
denotes |
A deletion of FGFR2 creating a chimeric IIIb/IIIc exon in a child with Apert syndrome. |
| TextSentencer_T2 |
87-98 |
Sentence |
denotes |
BACKGROUND: |
| TextSentencer_T3 |
99-384 |
Sentence |
denotes |
Signalling by fibroblast growth factor receptor type 2 (FGFR2) normally involves a tissue-specific alternative splice choice between two exons (IIIb and IIIc), which generates two receptor isoforms (FGFR2b and FGFR2c respectively) with differing repertoires of FGF-binding specificity. |
| TextSentencer_T4 |
385-524 |
Sentence |
denotes |
Here we describe a unique chimeric IIIb/c exon in a patient with Apert syndrome, generated by a non-allelic homologous recombination event. |
| TextSentencer_T5 |
525-543 |
Sentence |
denotes |
CASE PRESENTATION: |
| TextSentencer_T6 |
544-696 |
Sentence |
denotes |
We present a child with Apert syndrome in whom routine genetic testing had excluded the FGFR2 missense mutations commonly associated with this disorder. |
| TextSentencer_T7 |
697-901 |
Sentence |
denotes |
The patient was found to harbour a heterozygous 1372 bp deletion between FGFR2 exons IIIb and IIIc, apparently originating from recombination between 13 bp of identical DNA sequence present in both exons. |
| TextSentencer_T8 |
902-962 |
Sentence |
denotes |
The rearrangement was not present in the unaffected parents. |
| TextSentencer_T9 |
963-975 |
Sentence |
denotes |
CONCLUSIONS: |
| TextSentencer_T10 |
976-1112 |
Sentence |
denotes |
Based on the known pathogenesis of Apert syndrome, the chimeric FGFR2 protein is predicted to act in a dominant gain-of-function manner. |
| TextSentencer_T11 |
1113-1297 |
Sentence |
denotes |
This is likely to result from its expression in mesenchymal tissues, where retention of most of the residues essential for FGFR2b binding activity would result in autocrine activation. |
| TextSentencer_T12 |
1298-1449 |
Sentence |
denotes |
This report adds to the repertoire of rare cases of Apert syndrome for which a pathogenesis based on atypical FGFR2 rearrangements can be demonstrated. |
| T1 |
0-86 |
Sentence |
denotes |
A deletion of FGFR2 creating a chimeric IIIb/IIIc exon in a child with Apert syndrome. |
| T2 |
87-98 |
Sentence |
denotes |
BACKGROUND: |
| T3 |
99-384 |
Sentence |
denotes |
Signalling by fibroblast growth factor receptor type 2 (FGFR2) normally involves a tissue-specific alternative splice choice between two exons (IIIb and IIIc), which generates two receptor isoforms (FGFR2b and FGFR2c respectively) with differing repertoires of FGF-binding specificity. |
| T4 |
385-524 |
Sentence |
denotes |
Here we describe a unique chimeric IIIb/c exon in a patient with Apert syndrome, generated by a non-allelic homologous recombination event. |
| T5 |
525-543 |
Sentence |
denotes |
CASE PRESENTATION: |
| T6 |
544-696 |
Sentence |
denotes |
We present a child with Apert syndrome in whom routine genetic testing had excluded the FGFR2 missense mutations commonly associated with this disorder. |
| T7 |
697-901 |
Sentence |
denotes |
The patient was found to harbour a heterozygous 1372 bp deletion between FGFR2 exons IIIb and IIIc, apparently originating from recombination between 13 bp of identical DNA sequence present in both exons. |
| T8 |
902-962 |
Sentence |
denotes |
The rearrangement was not present in the unaffected parents. |
| T9 |
963-975 |
Sentence |
denotes |
CONCLUSIONS: |
| T10 |
976-1112 |
Sentence |
denotes |
Based on the known pathogenesis of Apert syndrome, the chimeric FGFR2 protein is predicted to act in a dominant gain-of-function manner. |
| T11 |
1113-1297 |
Sentence |
denotes |
This is likely to result from its expression in mesenchymal tissues, where retention of most of the residues essential for FGFR2b binding activity would result in autocrine activation. |
| T12 |
1298-1449 |
Sentence |
denotes |
This report adds to the repertoire of rare cases of Apert syndrome for which a pathogenesis based on atypical FGFR2 rearrangements can be demonstrated. |