Glycerol-3-phosphate dehydrogenase 1-like protein (GPD1L)

Glycerol-3-phosphate dehydrogenase 1-like protein (GPD1L) is a cytoplasmic enzyme that catalyzes the conversion of sn-glycerol 3-phosphate to glycerone phosphate and plays a regulatory role in cardiac electrophysiology by interacting with the cardiac sodium channel protein SCN5A (NaV1.5)[1][2]. Defects in GPD1L have been associated with Brugada syndrome type 2 and sudden infant death syndrome, primarily due to reduced cardiac sodium current resulting from altered interaction or redox state–dependent modulation of sodium channels[1][2][3]. GPD1L mutations can decrease peak sodium current and sodium channel membrane localization, ultimately predisposing to life-threatening arrhythmias and sudden cardiac death[2][3]. Despite its past implication as a Brugada syndrome gene, more recent genetic studies suggest that GPD1L is a modifier of sodium channel function rather than a definitive monogenic cause in most cases[2].

Other names

GPD1LKIAA0089GPD1-Lglycerol-3-phosphate dehydrogenase 1-like protein

Potentially targeted by drugs that modulate sodium channel function indirectly through regulatory protein interactions (specific approved drugs not identified)