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Primary immunodeficiency or monogenic inflammatory bowel disease v7.26 GNAI2 Achchuthan Shanmugasundram Tag Q3_24_promote_green was removed from gene: GNAI2.
Primary immunodeficiency or monogenic inflammatory bowel disease v7.26 GNAI2 Achchuthan Shanmugasundram reviewed gene: GNAI2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Primary immunodeficiency or monogenic inflammatory bowel disease v7.25 GNAI2 Achchuthan Shanmugasundram Source NHS GMS was added to GNAI2.
Source Expert Review Green was added to GNAI2.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Primary immunodeficiency or monogenic inflammatory bowel disease v7.17 GNAI2 Arina Puzriakova Classified gene: GNAI2 as Amber List (moderate evidence)
Primary immunodeficiency or monogenic inflammatory bowel disease v7.17 GNAI2 Arina Puzriakova Added comment: Comment on list classification: There is sufficient evidence to promote this gene to Green at the next GMS panel update. Immunodeficiency/immunodysregulation was the most common feature reported in patients with pathogenic GNAI2 variants, supporting inclusion on this panel.
Primary immunodeficiency or monogenic inflammatory bowel disease v7.17 GNAI2 Arina Puzriakova Gene: gnai2 has been classified as Amber List (Moderate Evidence).
Primary immunodeficiency or monogenic inflammatory bowel disease v7.16 GNAI2 Arina Puzriakova Publications for gene: GNAI2 were set to PMID: 39298586
Primary immunodeficiency or monogenic inflammatory bowel disease v7.15 GNAI2 Arina Puzriakova Tag Q3_24_promote_green tag was added to gene: GNAI2.
Primary immunodeficiency or monogenic inflammatory bowel disease v7.15 GNAI2 Arina Puzriakova reviewed gene: GNAI2: Rating: ; Mode of pathogenicity: None; Publications: 39298586; Phenotypes: ; Mode of inheritance: None
Primary immunodeficiency or monogenic inflammatory bowel disease v6.16 GNAI2 Dmitrijs Rots gene: GNAI2 was added
gene: GNAI2 was added to Primary immunodeficiency or monogenic inflammatory bowel disease. Sources: Literature
Mode of inheritance for gene: GNAI2 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: GNAI2 were set to PMID: 39298586
Phenotypes for gene: GNAI2 were set to Immunodefficiency with multisystemic presentation
Penetrance for gene: GNAI2 were set to unknown
Mode of pathogenicity for gene: GNAI2 was set to Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype - please provide details in the comments
Added comment: PMID: 39298586 showed 20 case with "multiorgan dysfunction, with a spectrum of birth defects involving brain, endocrine, skeletal, and other systems. Prominent immune dysregulation resulted from increased infection susceptibility—caused by impaired GPCR signaling for migration of T cells and neutrophils—and life-threatening autoimmunity with T cell hyperresponsiveness." and functional work.
Sources: Literature