Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000022282 | SCV000800077 | likely pathogenic | Deficiency of UDPglucose-hexose-1-phosphate uridylyltransferase | 2018-05-22 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000022282 | SCV004294305 | pathogenic | Deficiency of UDPglucose-hexose-1-phosphate uridylyltransferase | 2023-02-21 | criteria provided, single submitter | clinical testing | This sequence change disrupts the translational stop signal of the GALT mRNA. It is expected to extend the length of the GALT protein by 49 additional amino acid residues. This variant is not present in population databases (gnomAD no frequency). This protein extension has been observed in individual(s) with galactosemia (PMID: 15841485, 28065439). ClinVar contains an entry for this variant (Variation ID: 25336). This variant results in an extension of the GALT protein. Other variant(s) that result in a similarly extended protein product (p.*380Argext*49) have been determined to be pathogenic (PMID: 10649501, 15633893, 20213376, 34030713). This suggests that these extensions are likely to be disease-causing. For these reasons, this variant has been classified as Pathogenic. |