Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics Munich, |
RCV000001492 | SCV000608396 | pathogenic | Autosomal recessive hypophosphatemic bone disease | 2017-10-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001851549 | SCV002108016 | uncertain significance | not provided | 2024-04-05 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with leucine, which is neutral and non-polar, at codon 353 of the SLC34A3 protein (p.Arg353Leu). This variant is present in population databases (rs121918234, gnomAD 0.004%). This missense change has been observed in individual(s) with clinical features of SLC34A3-related conditions and/or hypophosphatemic rickets (PMID: 16358215, 34805638; Invitae). It has also been observed to segregate with disease in related individuals. ClinVar contains an entry for this variant (Variation ID: 1427). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is not expected to disrupt SLC34A3 protein function with a negative predictive value of 80%. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Mendelics | RCV000001492 | SCV002519771 | pathogenic | Autosomal recessive hypophosphatemic bone disease | 2022-05-04 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV000001492 | SCV005681746 | uncertain significance | Autosomal recessive hypophosphatemic bone disease | 2024-01-20 | criteria provided, single submitter | clinical testing | |
OMIM | RCV000001492 | SCV000021647 | pathogenic | Autosomal recessive hypophosphatemic bone disease | 2006-02-01 | no assertion criteria provided | literature only |