Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV005057216 | SCV005726905 | uncertain significance | not specified | 2024-11-08 | criteria provided, single submitter | clinical testing | Variant summary: ALOX12B c.1369T>C (p.Ser457Pro) results in a non-conservative amino acid change located in the Lipoxygenase, C-terminal domain (IPR013819) of the encoded protein sequence. Four of five in-silico tools predict a benign effect of the variant on protein function. The variant was absent in 224966 control chromosomes. The available data on variant occurrences in the general population are insufficient to allow any conclusion about variant significance. c.1369T>C has been reported in the literature in at least two compound heterozygous individuals affected with Lamellar Ichthyosis (e.g. Hotz_2021, Diociaiuti_2024). These reports do not provide unequivocal conclusions about association of the variant with Lamellar Ichthyosis. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publications have been ascertained in the context of this evaluation (PMID: 38588653, 33435499). ClinVar contains an entry for this variant (Variation ID: 995745). Based on the evidence outlined above, the variant was classified as uncertain significance. |
Institute for Human Genetics, |
RCV001289954 | SCV001477961 | pathogenic | Autosomal recessive congenital ichthyosis 2 | 2021-01-07 | no assertion criteria provided | clinical testing |