Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Illumina Laboratory Services, |
RCV000341107 | SCV000355787 | uncertain significance | Chédiak-Higashi syndrome | 2016-06-14 | criteria provided, single submitter | clinical testing | |
Genome Diagnostics Laboratory, |
RCV000341107 | SCV000743804 | likely benign | Chédiak-Higashi syndrome | 2017-11-01 | criteria provided, single submitter | clinical testing | |
Clinical Genetics DNA and cytogenetics Diagnostics Lab, |
RCV000341107 | SCV000745220 | likely benign | Chédiak-Higashi syndrome | 2017-06-28 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000341107 | SCV001596998 | likely benign | Chédiak-Higashi syndrome | 2024-01-31 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001699343 | SCV004039505 | likely benign | not specified | 2023-08-15 | criteria provided, single submitter | clinical testing | Variant summary: LYST c.2363+10dupT alters a nucleotide located at a position not widely known to affect splicing. 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 0.00059 in 281428 control chromosomes, predominantly at a frequency of 0.0011 within the Latino subpopulation in the gnomAD database. This frequency is equal to estimated for a pathogenic variant in LYST causing Chediak-Higashi Syndrome (0.0011 vs 0.0011), strongly suggesting that the variant is a benign polymorphism found primarily in populations of Latino origin. To our knowledge, no occurrence of c.2363+10dupT in individuals affected with Chediak-Higashi Syndrome and no experimental evidence demonstrating its impact on protein function have been reported. Four submitters have cited clinical-significance assessments for this variant to ClinVar after 2014 classified this variant as uncertain significance (n=1) and likely benign (n=3). Based on the evidence outlined above, the variant was classified as likely benign. |
Clinical Genetics, |
RCV001699343 | SCV001926050 | benign | not specified | no assertion criteria provided | clinical testing |