Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000157937 | SCV000207872 | likely pathogenic | not provided | 2012-06-21 | criteria provided, single submitter | clinical testing | A D119N missense change likely associated with a KRAS-related disorder was identified in the KRAS gene. It has not been published as a mutation, nor has it been reported as a benign polymorphism to our knowledge. The D119N amino acid substitution is non-conservative with a negatively charged residue (Asp) being replaced by a neutral residue (Asn) at a position in the protein that is highly conserved across species. Another mutation in a nearby codon (N116S) has been reported in association with Noonan syndrome and in vitro studies showed that it exhibits gain-of-function activity (Razzaque et al., 2012). Therefore, D119N is a strong candidate for a disease-causing mutation, however the possibility that it is a benign variant cannot be excluded.The variant is found in NOONAN panel(s). |
Baylor Genetics | RCV000850568 | SCV000992784 | likely pathogenic | Noonan syndrome 3; Autoimmune lymphoproliferative syndrome type 4; Acute myeloid leukemia; Cardiofaciocutaneous syndrome 2 | 2017-12-31 | criteria provided, single submitter | clinical testing | |
Undiagnosed Diseases Network, |
RCV001269478 | SCV001449491 | likely pathogenic | KRAS-related RASopathy | 2020-09-23 | criteria provided, single submitter | clinical testing | |
Genome |
RCV000999628 | SCV001156338 | not provided | Noonan syndrome 3 | no assertion provided | phenotyping only | Variant interpreted as Uncertain significance and reported on 09-18-2012 by Lab or GTR ID 1006. GenomeConnect-CFC International assertions are reported exactly as they appear on the patient-provided report from the testing laboratory. Registry team members make no attempt to reinterpret the clinical significance of the variant. |