Total submissions: 9
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Claritas Genomics | RCV000449519 | SCV000537848 | uncertain significance | Global developmental delay | 2017-02-21 | criteria provided, single submitter | clinical testing | |
Genetic Services Laboratory, |
RCV000502541 | SCV000596260 | uncertain significance | not specified | 2016-11-02 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV000764270 | SCV000895284 | uncertain significance | Microcephalic osteodysplastic primordial dwarfism type II | 2018-10-31 | criteria provided, single submitter | clinical testing | |
Baylor Genetics | RCV000764270 | SCV001523585 | uncertain significance | Microcephalic osteodysplastic primordial dwarfism type II | 2020-06-03 | criteria provided, single submitter | clinical testing | This variant was determined to be of uncertain significance according to ACMG Guidelines, 2015 [PMID:25741868]. |
Gene |
RCV001577090 | SCV001804415 | likely benign | not provided | 2020-07-06 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001577090 | SCV002141030 | uncertain significance | not provided | 2025-01-13 | criteria provided, single submitter | clinical testing | This sequence change replaces cysteine, which is neutral and slightly polar, with tyrosine, which is neutral and polar, at codon 215 of the PCNT protein (p.Cys215Tyr). This variant is present in population databases (rs138962786, gnomAD 0.04%). This variant has not been reported in the literature in individuals affected with PCNT-related conditions. ClinVar contains an entry for this variant (Variation ID: 397627). An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
ARUP Laboratories, |
RCV000764270 | SCV004562311 | likely benign | Microcephalic osteodysplastic primordial dwarfism type II | 2023-11-11 | criteria provided, single submitter | clinical testing | |
Ce |
RCV001577090 | SCV004811004 | likely benign | not provided | 2024-03-01 | criteria provided, single submitter | clinical testing | PCNT: BP4 |
Prevention |
RCV003401441 | SCV004112380 | uncertain significance | PCNT-related disorder | 2024-09-29 | no assertion criteria provided | clinical testing | The PCNT c.644G>A variant is predicted to result in the amino acid substitution p.Cys215Tyr. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.043% of alleles in individuals of European (Non-Finnish) descent in gnomAD. Although we suspect that this variant may be benign, at this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |