Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Genetics, |
RCV003994766 | SCV004812869 | uncertain significance | Combined immunodeficiency due to MALT1 deficiency | 2023-09-04 | criteria provided, single submitter | clinical testing | This sequence change in MALT1 is predicted to replace valine with alanine at codon 231, p.(Val231Ala). The valine residue is moderately conserved (100 vertebrates, UCSC), and is located in the Ig-like C2 domain. There is a moderate physicochemical difference between valine and alanine. The highest population minor allele frequency in the population database gnomAD v2.1 is 0.0008% (1/113,108 alleles) in the European non-Finnish population. To our knowledge, this variant has not been previously reported in the relevant scientific literature or databases. Computational evidence predicts a benign effect for the missense substitution (REVEL = 0.027). Based on the classification scheme RMH Modified ACMG/AMP Guidelines v1.6.1, this variant is classified as a VARIANT OF UNCERTAIN SIGNIFICANCE. Following criteria are met: PM2_Supporting, BP4 |