Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV003096022 | SCV005061676 | benign | Glanzmann thrombasthenia | 2024-04-16 | reviewed by expert panel | curation | The NM_000419.5(ITGA2B):c.1600+16G>T variant is an intronic variant that is not predicted by SpliceAI to impact splicing (Delta scores 0.00). In addition, it occurs at a nucleotide that is not conserved as shown by phyloP score of 0.2397 (BP7). The highest population minor allele frequency in gnomAD v4.0.0 is 0.002865 (250/75040alleles) in the African/African-American population, which is higher than the ClinGen PD VCEP threshold (>0.0024), and therefore meets this criterion (BA1). In summary this variant is classified as Benign for autosomal recessive Glanzmann thrombasthenia, with ACMG criteria applied as specified by the PD VCEP: BA1, BP7 |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV002266275 | SCV002548011 | uncertain significance | not specified | 2022-05-31 | criteria provided, single submitter | clinical testing | Variant summary: ITGA2B c.1600+16G>T alters a non-conserved nucleotide located close to a canonical splice site and therefore could affect mRNA splicing, leading to a significantly altered protein sequence. 4/4 computational tools predict no significant impact on normal splicing. However, these predictions have yet to be confirmed by functional studies. The variant allele was found at a frequency of 0.00018 in 251450 control chromosomes. To our knowledge, no occurrence of c.1600+16G>T in individuals affected with ITGA2B-Related Disorders and no experimental evidence demonstrating its impact on protein function have been reported. No clinical diagnostic laboratories have submitted clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as uncertain significance. |
Labcorp Genetics |
RCV003096022 | SCV002960868 | benign | Glanzmann thrombasthenia | 2024-01-04 | criteria provided, single submitter | clinical testing |