Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV000005411 | SCV001373859 | likely pathogenic | Tuberous sclerosis 1 | 2020-11-25 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. This variant has been reported to affect TSC1 protein function (PMID: 18830229, 21309039, 20547222). This variant has been observed in individuals and families affected with tuberous sclerosis complex (PMID: 18830229, Invitae). ClinVar contains an entry for this variant (Variation ID: 5104). This variant is not present in population databases (ExAC no frequency). This sequence change replaces methionine with arginine at codon 224 of the TSC1 protein (p.Met224Arg). The methionine residue is moderately conserved and there is a moderate physicochemical difference between methionine and arginine. |
OMIM | RCV000005411 | SCV000025593 | pathogenic | Tuberous sclerosis 1 | 2009-03-01 | no assertion criteria provided | literature only | |
Tuberous sclerosis database |
RCV000042336 | SCV000066125 | not provided | Tuberous sclerosis syndrome | no assertion provided | curation |