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Cardiomyopathy_Paediatric v1.49 NDUFA5 Sarah Milton Classified gene: NDUFA5 as Amber List (moderate evidence)
Cardiomyopathy_Paediatric v1.49 NDUFA5 Sarah Milton Gene: ndufa5 has been classified as Amber List (Moderate Evidence).
Cardiomyopathy_Paediatric v1.48 NDUFA5 Sarah Milton edited their review of gene: NDUFA5: Changed phenotypes: Mitochondrial disease, MONDO:0044970, NDUFA5-related
Cardiomyopathy_Paediatric v1.48 NDUFA5 Sarah Milton gene: NDUFA5 was added
gene: NDUFA5 was added to Cardiomyopathy_Paediatric. Sources: Literature
Mode of inheritance for gene: NDUFA5 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: NDUFA5 were set to 41916321
Phenotypes for gene: NDUFA5 were set to Mitochondrial disease, MONDO:0044970
Review for gene: NDUFA5 was set to AMBER
Added comment: PMID 41916321 reports 4 individuals from 3 unrelated families with biallelic loss-of-function NDUFA5 variants presenting with early onset multisystem mitochondrial disorder with complex I deficiency. One individual had hypertrophic cardiomyopathy and one had slight biventricular hypertrophy.

Paediatric onset HCM is likely to be a feature of this disorder given it is seen in other complex I deficiencies however few patients have been reported thus far.
Sources: Literature
Cardiomyopathy_Paediatric v0.231 Zornitza Stark removed gene:NDUFA5 from the panel
Cardiomyopathy_Paediatric v0.230 Zornitza Stark Copied gene NDUFA5 from panel Mitochondrial disease
Cardiomyopathy_Paediatric v0.230 NDUFA5 Zornitza Stark gene: NDUFA5 was added
gene: NDUFA5 was added to Cardiomyopathy_Paediatric. Sources: Expert Review Green,Literature
Mode of inheritance for gene: NDUFA5 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: NDUFA5 were set to 41916321
Phenotypes for gene: NDUFA5 were set to Complex I deficiency
Penetrance for gene: NDUFA5 were set to Complete