Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Undiagnosed Diseases Network, |
RCV000024225 | SCV000622153 | pathogenic | Amelocerebrohypohidrotic syndrome | 2015-12-23 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000024225 | SCV001583198 | pathogenic | Amelocerebrohypohidrotic syndrome | 2020-11-18 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in ROGDI are known to be pathogenic (PMID: 22424600, 23086778). This variant has been observed in individual(s) with Kohlschutter-Tonz syndrome (PMID: 22424600). ClinVar contains an entry for this variant (Variation ID: 31226). This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Gln96*) in the ROGDI gene. It is expected to result in an absent or disrupted protein product. |
OMIM | RCV000024225 | SCV000045516 | pathogenic | Amelocerebrohypohidrotic syndrome | 2006-02-01 | no assertion criteria provided | literature only |