Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV004586414 | SCV005077772 | uncertain significance | Leber optic atrophy | 2024-04-24 | criteria provided, single submitter | clinical testing | The m.14500A>T variant is not present in publicly available population databases like gnomAD v3.1, mtDB and our in-house exome database. The variant is present in MITOMAP and HelixMtdb databases at very low frequencies, only in heteroplasmic state. This variant has neither been published in literature with MT-ND6-related conditions nor reported to the ClinVar or OMIM databases, in any affected individuals. This variant is present in the MitImpact database (ID- MI.23822). In-silico pathogenicity prediction programs like SIFT4G, PolyPhen-2, CADD, APOGEE1 etc predicted this variant to be likely deleterious, however these predictions were not confirmed by published functional studies. |