ClinVar Miner

Submissions for variant NM_144997.7(FLCN):c.199dup (p.Ala67fs)

dbSNP: rs1555611438
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Total submissions: 3
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Submitter RCV SCV Clinical significance Condition Last evaluated Review status Method Comment
Ambry Genetics RCV005834484 SCV006526217 pathogenic Hereditary cancer-predisposing syndrome 2025-06-26 criteria provided, single submitter clinical testing The c.199dupG pathogenic mutation, located in coding exon 1 of the FLCN gene, results from a duplication of G at nucleotide position 199, causing a translational frameshift with a predicted alternate stop codon (p.A67Gfs*33). This variant was reported in individual(s) with features consistent with Birt-Hogg-Dube syndrome (Namba Y et al. PLoS One, 2023 Jul;18:e0289175). This variant is considered to be rare based on population cohorts in the Genome Aggregation Database (gnomAD). This alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. As such, this alteration is interpreted as a disease-causing mutation.
Center for Genomic Medicine, Rigshospitalet, Copenhagen University Hospital RCV003322322 SCV004026723 pathogenic not provided 2025-03-04 criteria provided, single submitter clinical testing
Division of Respiratory Medicine of Juntendo University, Juntendo University Faculty of Medicine and Graduate School of Medicine RCV003326170 SCV004032377 pathogenic Birt-Hogg-Dube syndrome 2023-07-01 no assertion criteria provided clinical testing

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