Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001825070 | SCV002074213 | uncertain significance | not specified | 2022-01-11 | criteria provided, single submitter | clinical testing | Variant summary: CHRNG c.1421G>A (p.Arg474His) results in a non-conservative amino acid change in the encoded protein sequence. Five of five in-silico tools predict a damaging effect of the variant on protein function. The variant allele was found at a frequency of 6.8e-05 in 250812 control chromosomes (gnomAD, exomes dataset). This frequency is not higher than expected for a pathogenic variant in CHRNG causing CHRNG Related Lethal Multiple Pterygium Syndrome (0.0011), allowing no conclusion about variant significance. To our knowledge, no occurrence of c.1421G>A in individuals affected with CHRNG Related Lethal Multiple Pterygium Syndrome and no experimental evidence demonstrating its impact on protein function have been reported. No clinical diagnostic laboratories have submitted clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as uncertain significance. |
Fulgent Genetics, |
RCV002482381 | SCV002778367 | uncertain significance | Autosomal recessive multiple pterygium syndrome; Lethal multiple pterygium syndrome | 2021-07-28 | criteria provided, single submitter | clinical testing |