Activity

Filter

Cancel
Date Panel Item Activity
3 actions
Cardiomyopathy_Paediatric v1.43 COQ2 Sarah Milton Classified gene: COQ2 as Green List (high evidence)
Cardiomyopathy_Paediatric v1.43 COQ2 Sarah Milton Gene: coq2 has been classified as Green List (High Evidence).
Cardiomyopathy_Paediatric v1.42 COQ2 Sarah Milton gene: COQ2 was added
gene: COQ2 was added to Cardiomyopathy_Paediatric. Sources: Literature
Mode of inheritance for gene: COQ2 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: COQ2 were set to 40929079; 33677064
Phenotypes for gene: COQ2 were set to Coenzyme Q10 deficiency, primary, 1, MIM#607426
Review for gene: COQ2 was set to GREEN
Added comment: Biallelic loss of function in COQ2 results in a wide spectrum of disease with the most severe being multisystem neonatal onset form with severity of presentation correlating to level of residual enzyme activity.

Hypertrophic cardiomyopathy has been reported in a small number of individuals with the severe neonatal form.

Note the small number of papers asserting a degree of treatment response to COQ10/4-hydroxybenzoic acid
Sources: Literature