Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000733105 | SCV000861124 | uncertain significance | not provided | 2018-05-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001237646 | SCV001410413 | uncertain significance | Nephronophthisis | 2022-05-30 | criteria provided, single submitter | clinical testing | 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. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Benign"; Align-GVGD: "Class C0"). ClinVar contains an entry for this variant (Variation ID: 597088). This variant has not been reported in the literature in individuals affected with NPHP3-related conditions. This variant is present in population databases (rs370506189, gnomAD 0.006%). This sequence change replaces valine, which is neutral and non-polar, with isoleucine, which is neutral and non-polar, at codon 911 of the NPHP3 protein (p.Val911Ile). |
Ambry Genetics | RCV003353014 | SCV004076121 | uncertain significance | Inborn genetic diseases | 2023-08-21 | criteria provided, single submitter | clinical testing | The c.2731G>A (p.V911I) alteration is located in exon 20 (coding exon 20) of the NPHP3 gene. This alteration results from a G to A substitution at nucleotide position 2731, causing the valine (V) at amino acid position 911 to be replaced by an isoleucine (I). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |