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Optic neuropathy v4.40 LHX2 Achchuthan Shanmugasundram Tag Q3_23_promote_green was removed from gene: LHX2.
Optic neuropathy v4.40 LHX2 Achchuthan Shanmugasundram reviewed gene: LHX2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Optic neuropathy v4.39 LHX2 Achchuthan Shanmugasundram Source NHS GMS was added to LHX2.
Source Expert Review Green was added to LHX2.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Optic neuropathy v4.33 LHX2 Arina Puzriakova Tag gene-checked tag was added to gene: LHX2.
Optic neuropathy v4.7 LHX2 Sarah Leigh edited their review of gene: LHX2: Changed rating: GREEN
Optic neuropathy v4.7 LHX2 Sarah Leigh Classified gene: LHX2 as Amber List (moderate evidence)
Optic neuropathy v4.7 LHX2 Sarah Leigh Added comment: Comment on list classification: There is enough evidence for this gene to be rated GREEN at the next major review.
Optic neuropathy v4.7 LHX2 Sarah Leigh Gene: lhx2 has been classified as Amber List (Moderate Evidence).
Optic neuropathy v4.6 LHX2 Sarah Leigh Tag Q3_23_promote_green tag was added to gene: LHX2.
Optic neuropathy v4.6 LHX2 Sarah Leigh changed review comment from: Not associated with a phenotype in OMIM, Gen2Phen or MONDO. PMID: 37057675 reports 17 predominantly de novo LHX2 variants in a panel of patients with a variable neurodevelopmental disorder. Haploinsufficiency and functional studies are supportive of a loss-of-function pathogenic action of the reported LHX2 variants.
Seven out of the ten cases reported in table 1 (PMID: 37057675) are listed as having microcephaly, however, due to lack of clinical information, these cases cannot be classified as severe (personal communication with the author, Christiane Zweier).
Sources: Literature; to: Not associated with a phenotype in OMIM, Gen2Phen or MONDO. PMID: 37057675 reports 17 predominantly de novo LHX2 variants in a panel of patients with a variable neurodevelopmental disorder. Haploinsufficiency and functional studies are supportive of a loss-of-function pathogenic action of the reported LHX2 variants.
Nine out of the thirteen cases reported in table 1 (PMID: 37057675) are listed as having Ophthalmologic abnormalities including macular degeneration, optic neuropathy, and esotropia.
Sources: Literature
Optic neuropathy v4.6 LHX2 Sarah Leigh Entity copied from Severe microcephaly v4.28
Optic neuropathy v4.6 LHX2 Sarah Leigh gene: LHX2 was added
gene: LHX2 was added to Optic neuropathy. Sources: Expert Review Amber,Literature
Mode of inheritance for gene: LHX2 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Publications for gene: LHX2 were set to 37057675
Phenotypes for gene: LHX2 were set to neurodevelopmental disorder