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Progressive cardiac conduction disease v2.15 POPDC2 Achchuthan Shanmugasundram changed review comment from: Comment on list classification: There are five unrelated families reported with cardiac conduction disease. Hence, this gene can be promoted to green rating in the next GMS update.; to: Comment on list classification: There are five unrelated families reported with cardiac conduction disease and with biallelic POPDC2 variants. Hence, this gene can be promoted to green rating in the next GMS update.
Progressive cardiac conduction disease v2.15 POPDC2 Achchuthan Shanmugasundram Classified gene: POPDC2 as Amber List (moderate evidence)
Progressive cardiac conduction disease v2.15 POPDC2 Achchuthan Shanmugasundram Added comment: Comment on list classification: There are five unrelated families reported with cardiac conduction disease. Hence, this gene can be promoted to green rating in the next GMS update.
Progressive cardiac conduction disease v2.15 POPDC2 Achchuthan Shanmugasundram Gene: popdc2 has been classified as Amber List (Moderate Evidence).
Progressive cardiac conduction disease v2.14 POPDC2 Achchuthan Shanmugasundram gene: POPDC2 was added
gene: POPDC2 was added to Progressive cardiac conduction disease. Sources: Literature
Q2_26_promote_green tags were added to gene: POPDC2.
Mode of inheritance for gene: POPDC2 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: POPDC2 were set to 40409267; 41456958
Phenotypes for gene: POPDC2 were set to Cardiac conduction disease with or without cardiomyopathy 2, OMIM:621367; cardiac conduction disease with or without cardiomyopathy 2, MONDO:0700389
Review for gene: POPDC2 was set to GREEN
Added comment: PMID:40409267 (2025) reported the identification of biallelic variants in POPDC2 gene in six patients from four families presenting with a phenotypic spectrum consisting of sinus node dysfunction, AV conduction defects, and hypertrophic cardiomyopathy. Modelling and electrophysiological studies suggested effects of identified POPDC2 variants on cAMP binding and TREK-1 current.

PMID:41456958 (2026) reported the identification of a novel homozygous POPDC2 variant in a patient presenting with early-onset cardiac conduction disease and hypertrophic cardiomyopathy.

This gene has been associated with relevant phenotype in OMIM (MIM #621367, record last accessed on 22 April 2026), but not yet in Gene2Phenotype or ClinGen.
Sources: Literature