Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000022340 | SCV000797091 | uncertain significance | Renal carnitine transport defect | 2018-01-11 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000022340 | SCV001208278 | pathogenic | Renal carnitine transport defect | 2023-11-21 | criteria provided, single submitter | clinical testing | This sequence change replaces glycine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 242 of the SLC22A5 protein (p.Gly242Val). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with primary carnitine deficiency (PMID: 11058897). ClinVar contains an entry for this variant (Variation ID: 25387). 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 expected to disrupt SLC22A5 protein function with a positive predictive value of 95%. Experimental studies have shown that this missense change affects SLC22A5 function (PMID: 11058897). For these reasons, this variant has been classified as Pathogenic. |
Genome- |
RCV000022340 | SCV002055754 | likely pathogenic | Renal carnitine transport defect | 2021-07-15 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV000022340 | SCV005056773 | likely pathogenic | Renal carnitine transport defect | 2024-03-01 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV000022340 | SCV005669209 | likely pathogenic | Renal carnitine transport defect | 2024-05-07 | criteria provided, single submitter | clinical testing |