Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004703019 | SCV005202305 | likely pathogenic | Vitamin D-dependent rickets, type 1A | 2024-07-29 | criteria provided, single submitter | clinical testing | Variant summary: CYP27B1 c.374G>T (p.Gly125Val) 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 was absent in 177120 control chromosomes. To our knowledge, no occurrence of c.374G>T in individuals affected with Vitamin D-dependent rickets has been reported. At least one publication reports experimental evidence evaluating an impact on protein function. The most pronounced variant effect results in <10% of normal activity from G125E/A/V mutants (Sawada_2001). The following publication has been ascertained in the context of this evaluation (PMID: 11737215). No submitters have cited clinical-significance assessments for this variant to ClinVar. Based on the evidence outlined above, the variant was classified as likely pathogenic. |