Total submissions: 8
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002453678 | SCV002613070 | uncertain significance | Cardiovascular phenotype | 2020-06-23 | criteria provided, single submitter | clinical testing | The p.P11704Q variant (also known as c.35111C>A), located in coding exon 131 of the TTN gene, results from a C to A substitution at nucleotide position 35111. The proline at codon 11704 is replaced by glutamine, an amino acid with similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, the in silico prediction for this alteration is inconclusive. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Baylor Genetics | RCV003147389 | SCV003835204 | uncertain significance | Early-onset myopathy with fatal cardiomyopathy | 2022-10-13 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV003147386 | SCV003835505 | uncertain significance | Tibial muscular dystrophy | 2022-10-13 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV003147387 | SCV003835701 | uncertain significance | Autosomal recessive limb-girdle muscular dystrophy type 2J | 2022-10-13 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV003147384 | SCV003835702 | uncertain significance | Hypertrophic cardiomyopathy 9 | 2022-10-13 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV003147388 | SCV003835802 | uncertain significance | Myopathy, myofibrillar, 9, with early respiratory failure | 2022-10-13 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV003147385 | SCV003835856 | uncertain significance | Dilated cardiomyopathy 1G | 2022-10-13 | criteria provided, single submitter | clinical testing | |
Gene |
RCV000184690 | SCV000237385 | not provided | not provided | 2013-11-18 | no assertion provided | clinical testing | Missense variants in the TTN gene are considered 'unclassified' if they are not previously reported in the literature and do not have >1% frequency in the population to be considered a polymorphism. Research indicates that truncating mutations in the TTN gene are expected to account for approximately 25% of familial and 18% of sporadic idiopathic DCM; however, truncating variants in the TTN gene have been reported in approximately 3% of reported control alleles. There has been little investigation into non-truncating variants. (Herman D et al. Truncations of titin causing dilated cardiomyopathy. N Eng J Med 366:619-628, 2012) The variant is found in DCM-CRDM panel(s). |