Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000478244 | SCV000567333 | pathogenic | not provided | 2015-09-08 | criteria provided, single submitter | clinical testing | The R609X nonsense variant in the FGFR1 gene has been reported previously inassociation with Kallmann syndrome (Riley et al., 2007; Marcos et al., 2014). This variant is predicted to cause loss of normal protein function either through protein truncation ornonsense-mediated mRNA decay. Therefore, we consider the R609X variant to be pathogenic. |
Genetic Services Laboratory, |
RCV000500417 | SCV000594769 | pathogenic | Hypogonadotropic hypogonadism 2 with or without anosmia | 2017-04-28 | criteria provided, single submitter | clinical testing | |
Reproductive Endocrine Unit, |
RCV000500417 | SCV003932494 | pathogenic | Hypogonadotropic hypogonadism 2 with or without anosmia | 2023-05-04 | criteria provided, single submitter | research | The variant NM_023110.2:c.1825C>T, p.(Arg609*) het has been classified as P1c based on the variant meeting the following ACMG Criteria: PVS1,PM2,PP3. |
Labcorp Genetics |
RCV003764580 | SCV004569644 | pathogenic | Hypogonadotropic hypogonadism 2 with or without anosmia; Pfeiffer syndrome | 2023-07-27 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg609*) in the FGFR1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in FGFR1 are known to be pathogenic (PMID: 12627230). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with clinical features of Kallmann syndrome (PMID: 17360555). ClinVar contains an entry for this variant (Variation ID: 16296). For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV000030934 | SCV000037966 | risk factor | Hypogonadotropic hypogonadism 2 with anosmia | 2007-03-13 | no assertion criteria provided | literature only | |
Genomics England Pilot Project, |
RCV000500417 | SCV001760211 | pathogenic | Hypogonadotropic hypogonadism 2 with or without anosmia | no assertion criteria provided | clinical testing |