c.3811C>T (p.Arg1271X)
Variant ID | Exon |
60 | Exon 31 |
RNA effect | EPP | Control frequency | Controls origin |
RNA stability | 3 | 0/94 | Central Europe, Middle East, East Asia and US |
The reading frame is interrupted by a premature STOP codon.
The mRNA produced might be targeted for nonsense mediated decay (NMD).
Patient information | Allele 1 | Allele 2 | |||||||||||||||
Patient ID | Family ID | Gender | Age | Origin | Disease | Segregation | Parental consanguinity | Consanguinity degree | Exon/intron | Nucleotide nomencl. | Protein nomencl. | Detection method | Exon/intron | Nucleotide nomencl. | Protein nomencl. | Detection method | Reference |
COR004a | COR004 | female | 17 y | Italy | CORS | yes | no | Exon 31 | 3811C>T | R1271X | DHPLC | Exon 42 | 5734delT | R1911fsX1922 | DHPLC | Brancati (2007) | |
COR004b | COR004 | male | 7 y | Italy | CORS | yes | no | Exon 31 | 3811C>T | R1271X | DHPLC | Exon 42 | 5734delT | R1911fsX1922 | DHPLC | Brancati (2007) | |
A372 II-1 | A372 | Italy | CORS | yes | no | Exon 32 | C3811T | R1271X | DHPLC | Exon 43 | 5734delT | W1912fsX1923 | DHPLC | Helou (2007) | |||
A372 II-2 | A372 | Italy | CORS | yes | no | Exon 32 | C3811T | R1271X | DHPLC | Exon 43 | 5734delT | W1912fsX1923 | DHPLC | Helou (2007) | |||
5 | S5 | LCA | unknown | unknown | Exon 31 | CGA>TGA | Arg1271STOP | Direct sequencing (Sanger) | Stone (2007) |