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Mendeliome v1.4668 FXYD2 Sangavi Sivagnanasundram changed review comment from: Classified as MODERATE by ClinGen Tubulopathy VCEP 29/06/2023 - https://search.clinicalgenome.org/CCID:004896 - this is based of the reports of a singular variant in multiple families

PMID: 40428357 - reports a Polish family (2 sibs and mother) presenting with hypercalciuria, glucosuria and mild proteinuria. The mother presented with a milder phenotype compared to the children.
c.80G>A, p.(Arg27His) - Variant is present in gnomAD v4.1 with global FAF - 0.00098% (23 hets globally)

Upgrade to green given a second variant that is shown to segregate in affected members of the family (siblings and mother); to: Classified as MODERATE by ClinGen Tubulopathy VCEP 29/06/2023 - https://search.clinicalgenome.org/CCID:004896 - this is based of the reports of a singular variant in multiple families

PMID: 40428357 - reports a Polish family (2 sibs and mother) presenting with hypercalciuria, glucosuria and mild proteinuria. The mother presented with a milder phenotype compared to the children.
c.80G>A, p.(Arg27His) - Variant is present in gnomAD v4.1 with global FAF - 0.00098% (23 hets globally)

Remain as Amber given that only two variants have been reported in affected individuals and no supportive functional evidence has been reported as of yet.
Mendeliome v1.4668 FXYD2 Sangavi Sivagnanasundram edited their review of gene: FXYD2: Changed rating: AMBER
Mendeliome v1.4668 FXYD2 Sangavi Sivagnanasundram reviewed gene: FXYD2: Rating: GREEN; Mode of pathogenicity: None; Publications: 40428357; Phenotypes: Renal hypomagnesemia 2, MONDO:0007937; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Mendeliome v0.14244 FXYD2 Bryony Thompson Marked gene: FXYD2 as ready
Mendeliome v0.14244 FXYD2 Bryony Thompson Gene: fxyd2 has been classified as Amber List (Moderate Evidence).
Mendeliome v0.14244 FXYD2 Bryony Thompson Phenotypes for gene: FXYD2 were changed from to Renal hypomagnesemia 2 MONDO:0007937
Mendeliome v0.14243 FXYD2 Bryony Thompson Publications for gene: FXYD2 were set to
Mendeliome v0.14242 FXYD2 Bryony Thompson Mode of pathogenicity for gene: FXYD2 was changed from to Other
Mendeliome v0.14241 FXYD2 Bryony Thompson Mode of inheritance for gene: FXYD2 was changed from Unknown to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Mendeliome v0.14240 FXYD2 Bryony Thompson Classified gene: FXYD2 as Amber List (moderate evidence)
Mendeliome v0.14240 FXYD2 Bryony Thompson Gene: fxyd2 has been classified as Amber List (Moderate Evidence).
Mendeliome v0.14239 FXYD2 Bryony Thompson reviewed gene: FXYD2: Rating: AMBER; Mode of pathogenicity: Other; Publications: 17980699, 12763862, 18448590, 11062458, 25765846, 27014088; Phenotypes: Renal hypomagnesemia 2 MONDO:0007937; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Mendeliome v0.0 FXYD2 Zornitza Stark gene: FXYD2 was added
gene: FXYD2 was added to Mendeliome_VCGS. Sources: Expert Review Green,Victorian Clinical Genetics Services
Mode of inheritance for gene: FXYD2 was set to Unknown