Mitochondrial potassium channel (CCDC51) (CCDC51)

Mitochondrial potassium channel (CCDC51), also known as MITOK, is a coiled-coil domain-containing protein encoded by the CCDC51 gene on chromosome 3. It serves as the pore-forming subunit of the mitochondrial ATP-gated potassium channel (mitoK(ATP)), operating in a complex with the ATP-binding ABCB8 (mitochondrial sulfonylurea receptor, MITOSUR). This channel is localized exclusively to the inner mitochondrial membrane. CCDC51 enables ATP-regulated potassium ion flux into mitochondria, helping to regulate mitochondrial matrix volume, membrane potential, and oxidative phosphorylation, especially during cellular stress. Dysregulation of this channel can result in mitochondrial dysfunction, altered cellular metabolism, and compromised organ protection mechanisms, particularly under conditions such as ischemia-reperfusion. Experimental knockout of CCDC51 in animal models leads to defects in cardiac protection and vision (retinopathy) due to photoreceptor degeneration. While channel modulators like glibenclamide can inhibit the mitoK(ATP) channel, CCDC51 itself currently has no specific clinical drugs targeting it directly. Broader roles in human disease remain under study, and CCDC51 is mainly of interest as a potential therapeutic target for modulating mitochondrial stress responses and cell survival

Other names

MITOKCoiled-coil domain-containing protein 51FLJ12436Mitochondrial potassium channel, CCDC51MITOK, mitochondrial potassium channel

Drugs that modulate mitochondrial ATP-dependent potassium channels, such as glibenclamide, act by blocking mitochondrial K+ flux, thereby modulating mitochondrial membrane potential, volume, and downstream responses to metabolic stress or ischemia-reperfusion