Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV003479578 | SCV004223013 | uncertain significance | not specified | 2023-11-21 | criteria provided, single submitter | clinical testing | Variant summary: POU3F4 c.359C>A (p.Pro120Gln) results in a non-conservative amino acid change located in the POU-specific domain (IPR000327) of the encoded protein sequence. Four of five in-silico tools predict a benign effect of the variant on protein function. The variant allele was found at a frequency of 2.2e-05 in 178362 control chromosomes (gnomAD). The available data on variant occurrences in the general population are insufficient to allow any conclusion about variant significance. To our knowledge, no occurrence of c.359C>A in individuals affected with Nonsyndromic Hearing Loss And Deafness, X-Linked 2 and no experimental evidence demonstrating its impact on protein function have been reported. No submitters have cited 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 |
RCV003549106 | SCV004270660 | benign | not provided | 2023-08-14 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004364825 | SCV005007612 | uncertain significance | Inborn genetic diseases | 2022-12-23 | criteria provided, single submitter | clinical testing | The c.359C>A (p.P120Q) alteration is located in exon 1 (coding exon 1) of the POU3F4 gene. This alteration results from a C to A substitution at nucleotide position 359, causing the proline (P) at amino acid position 120 to be replaced by a glutamine (Q). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Prevention |
RCV003919246 | SCV004729152 | likely benign | POU3F4-related disorder | 2023-06-22 | no assertion criteria provided | clinical testing | This variant is classified as likely benign based on ACMG/AMP sequence variant interpretation guidelines (Richards et al. 2015 PMID: 25741868, with internal and published modifications). |