Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001327457 | SCV001518532 | uncertain significance | Nephronophthisis 9 | 2020-03-05 | criteria provided, single submitter | clinical testing | This sequence change replaces threonine with methionine at codon 667 of the NEK8 protein (p.Thr667Met). The threonine residue is moderately conserved and there is a moderate physicochemical difference between threonine and methionine. This variant is present in population databases (rs147625932, ExAC 0.003%). 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 output the following: SIFT: "Tolerated"; PolyPhen-2: "Benign"; Align-GVGD: "Class C0". The methionine amino acid residue is found in multiple mammalian species, suggesting that this missense change does not adversely affect protein function. These predictions have not been confirmed by published functional studies and their clinical significance is uncertain. This variant has not been reported in the literature in individuals with NEK8-related conditions. |
Fulgent Genetics, |
RCV002504515 | SCV002814145 | uncertain significance | Nephronophthisis 9; Renal-hepatic-pancreatic dysplasia 2 | 2022-01-02 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002546224 | SCV003616198 | uncertain significance | Inborn genetic diseases | 2022-06-24 | criteria provided, single submitter | clinical testing | The c.2000C>T (p.T667M) alteration is located in exon 14 (coding exon 14) of the NEK8 gene. This alteration results from a C to T substitution at nucleotide position 2000, causing the threonine (T) at amino acid position 667 to be replaced by a methionine (M). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |