Cardiolipin-containing mitochondrial membrane (CL)

Cardiolipin is a unique dimeric phospholipid located primarily within the inner mitochondrial membrane, where it is essential for the structural integrity and functional efficiency of the mitochondria [1]. It facilitates the assembly of respiratory chain supercomplexes and maintains the curvature of cristae, which are vital for optimal ATP synthesis [3]. Pathological alterations in cardiolipin, such as its peroxidation or the accumulation of immature forms (as seen in Barth syndrome), lead to mitochondrial decay, electron leakage, and the triggering of cell death pathways [1][3]. Therapeutic strategies targeting cardiolipin-containing membranes involve small molecules or peptides, such as elamipretide, that bind to cardiolipin to stabilize its interaction with proteins like cytochrome c [2]. By preserving the mitochondrial membrane architecture, these interventions aim to reduce oxidative stress and restore energy production in diseases characterized by mitochondrial dysfunction, including heart failure and various rare genetic disorders [4]. Sources: [1] Paradies G, et al. (2014). Cardiolipin: a heart of mitochondrial function and dysfunction. Trends in Endocrinology & Metabolism. [2] Szeto HH. (2014). First-in-class cardiolipin-protective compound as a therapeutic agent for mitochondrial dysfunction. Antioxidants & Redox Signaling. [3] Chicco AJ, Sparagna GC. (2007). Role of cardiolipin alterations in mitochondrial dysfunction and disease. American Journal of Physiology-Cell Physiology. [4] Chatfield KC, et al. (2022). Elamipretide Retains Muscle Function in Barth Syndrome. Genetics in Medicine.

Other names

Diphosphatidylglycerol1,3-bis(sn-3'-phosphatidyl)-sn-glycerolMitochondrial cardiolipinInner mitochondrial membrane cardiolipin

Stabilization of cardiolipin-cytochrome c complexes and prevention of cardiolipin peroxidation to maintain mitochondrial cristae structure and respiratory chain efficiency.