Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001237931 | SCV001410722 | pathogenic | RASopathy | 2019-10-25 | criteria provided, single submitter | clinical testing | This variant has been reported to affect MAP2K1 protein function (PMID: 19915144). For these reasons, this variant has been classified as Pathogenic. This variant has been observed in individual(s) with clinical features of Noonan syndrome (Invitae). In at least one individual the variant was observed to be de novo. ClinVar contains an entry for this variant (Variation ID: 376194). This variant is not present in population databases (ExAC no frequency). This sequence change replaces isoleucine with asparagine at codon 111 of the MAP2K1 protein (p.Ile111Asn). The isoleucine residue is highly conserved and there is a large physicochemical difference between isoleucine and asparagine. |