Cardiolipin synthase 1 (CRLS1)

Cardiolipin synthase 1 (CRLS1) is a mitochondrial inner membrane enzyme responsible for the final step in cardiolipin biosynthesis, catalyzing the transfer of a phosphatidyl group from CDP-diacylglycerol to phosphatidylglycerol to produce cardiolipin, a unique dimeric phospholipid essential for mitochondrial function[1][3][4][5]. Cardiolipin is critical for the stability and activity of protein complexes in the mitochondrial respiratory chain, for maintaining membrane dynamics and curvature, and for cellular processes such as mitophagy, energy metabolism, and apoptosis[2][4][5]. CRLS1 is highly expressed in tissues with high mitochondrial energy demand, such as skeletal and cardiac muscle, liver, pancreas, and kidney[3][4]. Loss or dysfunction of CRLS1 impairs cardiolipin synthesis, leading to mitochondrial dysfunction, reduced ATP production, and pathologies including muscle degeneration, neurodegeneration, and inherited mitochondrial diseases[1][3][4]. CRLS1 and cardiolipin biosynthesis are increasingly recognized as potential therapeutic targets in metabolic diseases, sarcopenia (age-related muscle atrophy), and mitochondrial myopathies, and are the focus of experimental drug development and biomarker research[4].

Other names

Cardiolipin synthase (CMP-forming)C20orf155CLS1CLSdJ967N21.6GCD10Protein GCD10 homologGCD10 homolog (S. cerevisiae)COSPD57

Enhancement of cardiolipin synthesis or stabilization to improve mitochondrial function