Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001928006 | SCV002177968 | uncertain significance | Deficiency of alpha-mannosidase | 2022-10-17 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with cysteine, which is neutral and slightly polar, at codon 308 of the MAN2B1 protein (p.Arg308Cys). This variant is present in population databases (rs185112259, gnomAD 0.05%). This variant has not been reported in the literature in individuals affected with MAN2B1-related conditions. ClinVar contains an entry for this variant (Variation ID: 1410243). 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) has been performed at Invitae for this missense variant, however the output from this modeling did not meet the statistical confidence thresholds required to predict the impact of this variant on MAN2B1 protein function. 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. |
Mayo Clinic Laboratories, |
RCV002261418 | SCV002541499 | uncertain significance | not provided | 2021-10-26 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002555780 | SCV003676106 | uncertain significance | Inborn genetic diseases | 2021-08-03 | criteria provided, single submitter | clinical testing | The c.922C>T (p.R308C) alteration is located in exon 7 (coding exon 7) of the MAN2B1 gene. This alteration results from a C to T substitution at nucleotide position 922, causing the arginine (R) at amino acid position 308 to be replaced by a cysteine (C). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |