Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Pele Pequeno Principe Research Institute, |
RCV001174526 | SCV001132036 | pathogenic | Oculocutaneous albinism type 1B | criteria provided, single submitter | research | ||
Genome- |
RCV001593159 | SCV001821965 | pathogenic | Tyrosinase-negative oculocutaneous albinism | 2021-07-22 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001858606 | SCV002247388 | pathogenic | not provided | 2023-07-20 | criteria provided, single submitter | clinical testing | This sequence change affects an acceptor splice site in intron 3 of the TYR gene. It is expected to disrupt RNA splicing. Variants that disrupt the donor or acceptor splice site typically lead to a loss of protein function (PMID: 16199547), and loss-of-function variants in TYR are known to be pathogenic (PMID: 23504663). This variant is not present in population databases (gnomAD no frequency). Disruption of this splice site has been observed in individual(s) with ocular albinism and/or oculocutaneous albinism (PMID: 32411182; Invitae). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. ClinVar contains an entry for this variant (Variation ID: 800543). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. For these reasons, this variant has been classified as Pathogenic. |
Baylor Genetics | RCV004569822 | SCV005054565 | pathogenic | SKIN/HAIR/EYE PIGMENTATION 3, LIGHT/DARK SKIN | 2024-03-10 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV005004473 | SCV005631381 | likely pathogenic | Tyrosinase-negative oculocutaneous albinism; Oculocutaneous albinism type 1B; SKIN/HAIR/EYE PIGMENTATION 3, LIGHT/DARK SKIN | 2024-04-12 | criteria provided, single submitter | clinical testing |