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Likely inborn error of metabolism v7.25 IDH1 Sarah Leigh edited their review of gene: IDH1: Added comment: Numerous reports of IDH1 variants associated with OMIM:614875, OMIM:614569 and OMIM:166000 (PMID: 24049096; 22025298; 22057234; 22057236).; Changed rating: GREEN
Likely inborn error of metabolism v7.25 IDH1 Sarah Leigh Tag mosaicism tag was added to gene: IDH1.
Tag Q2_25_ promote_green tag was added to gene: IDH1.
Likely inborn error of metabolism v7.25 IDH1 Sarah Leigh Classified gene: IDH1 as Amber List (moderate evidence)
Likely inborn error of metabolism v7.25 IDH1 Sarah Leigh Added comment: Comment on list classification: Somatic variants relevant to this gene, potentially resulting in mosaicism
Likely inborn error of metabolism v7.25 IDH1 Sarah Leigh Gene: idh1 has been classified as Amber List (Moderate Evidence).
Likely inborn error of metabolism v7.24 IDH1 Sarah Leigh Publications for gene: IDH1 were set to 33340416
Likely inborn error of metabolism v7.23 IDH1 Sarah Leigh Added comment: Comment on mode of inheritance: Somatic variants thought to occur early in development, resulting in mosaicism
Likely inborn error of metabolism v7.23 IDH1 Sarah Leigh Mode of inheritance for gene: IDH1 was changed from BIALLELIC, autosomal or pseudoautosomal to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Likely inborn error of metabolism v7.22 IDH1 Sarah Leigh Phenotypes for gene: IDH1 were changed from Failure to thrive; Psychomotor delay; Feeding difficulties; Increased D-2-Hydroxyglutaric acid in urine to Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria OMIM:614875; metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria, MONDO:0013941; Maffucci syndrome, OMIM:614569; Maffucci syndrome, MONDO:0013808; Ollier disease/ Dyschondroplasia, OMIM:166000; Ollier disease, MONDO:0008145
Likely inborn error of metabolism v2.256 IDH1 Sarah Leigh Publications for gene: IDH1 were set to PMID: 33340416
Likely inborn error of metabolism v2.154 IDH1 Andžela Lazdāne gene: IDH1 was added
gene: IDH1 was added to Inborn errors of metabolism. Sources: Literature
Mode of inheritance for gene: IDH1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: IDH1 were set to PMID: 33340416
Phenotypes for gene: IDH1 were set to Failure to thrive; Psychomotor delay; Feeding difficulties; Increased D-2-Hydroxyglutaric acid in urine
Review for gene: IDH1 was set to AMBER
Added comment: Isocitrate dehydrogenase 1 deficiency.

IEM Nosology Group (IEMbase):Disorders of the Krebs cycle. The IDH1 gene is included in International classification of inherited metabolic disorders (ICIMD), Disorders of the Krebs cycle.
Sources: Literature