Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000019314 | SCV001388202 | uncertain significance | Hypoproteinemia, hypercatabolic | 2021-09-01 | criteria provided, single submitter | clinical testing | This sequence change replaces alanine with proline at codon 11 of the B2M protein (p.Ala11Pro). The alanine residue is weakly conserved and there is a small physicochemical difference between alanine and proline. This variant is not present in population databases (ExAC no frequency). This missense change has been observed in individual(s) with familial hypercatabolic hypoproteinemia (PMID: 16549777). It has also been observed to segregate with disease in related individuals. This variant is also known as G913C. ClinVar contains an entry for this variant (Variation ID: 17740). Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. 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. |
OMIM | RCV000019314 | SCV000039603 | pathogenic | Hypoproteinemia, hypercatabolic | 2006-03-28 | no assertion criteria provided | literature only |