Stomatin-like protein 2 (SLP-2) is a widely expressed mitochondrial inner membrane protein of unknown function. Here we show that human SLP-2 interacts with prohibitin-1 and -2 and binds to the mitochondrial membrane phospholipid cardiolipin. Upregulation of SLP-2 expression increases cardiolipin content and the formation of metabolically active mitochondrial membranes and induces mitochondrial...

Cardiolipin is a mitochondria-specific phospholipid known to be intimately involved with apoptosis. However, the lack of appropriate cellular models to date restricted analysis of its role in cell death. The maturation of cardiolipin requires the transacylase tafazzin, which is mutated in the human disorder Barth syndrome. Using Barth syndrome patient-derived cells and HeLa cells in which tafaz...

The object of this study was to investigate whether the levels of cardiolipin in cultured skin fibroblasts of patients with Barth syndrome (BTHS) can be restored by addition of linoleic acid to growth media. To this end, fibroblasts from controls and BTHS patients were grown in the presence or absence of linoleic acid. High-performance liquid chromatography-electrospray ionization tandem mass s...

Bacterial membranes are highly organized, containing specific microdomains that facilitate distinct protein and lipid assemblies. Evidence suggests that cardiolipin molecules segregate into such microdomains, probably conferring a negative curvature to the inner plasma membrane during membrane fission upon cell division. 3',6-Dinonyl neamine is an amphiphilic aminoglycoside derivative active ag...

The phospholipid, cardiolipin, is essential for maintaining mitochondrial structure and optimal function. Cardiolipin-deficiency in humans, Barth syndrome, is characterized by exercise intolerance, dilated cardiomyopathy, neutropenia, and 3-methyl-glutaconic aciduria. The causative gene is the mitochondrial acyl-transferase, tafazzin, that is essential for remodeling acyl chains of cardiolipin....

In a previous communication (Shaw, 1955), I referred to several papers concerning the value of cardiolipin antigen in complement-fixation and flocculation tests. The majority of workers have reported increased sensitivity and specificity with this antigen, which has also been recommended to obtain reproducible results in various laboratories in different countries. At that time, I compared the ...

Although observations have implied that lupus anticoagulants have immunologic specificity toward anionic phospholipids. this assumption has been directly demonstrated in only one patient with a monoclonal 1gM paraprotein. We tested the generality of this hypothesis directly by isolating five lgG lupus anticoagulants from patients with lupuslike syndromes and/or thrombosis. lgG lupus anticoagula...

The transversal distribution of phosphatidylcholine, phosphatidylethanolamine, and cardiolipin in the inner membrane of beef heart mitochondria was investigated. Highly purified “right-side-out” (mitoplasts) and “inside-out” (submitochondrial particles) membranes were used, permitting the independent study of the phospholipid composition on either half of the membrane. Three independent, and pa...

Cardiolipin is an ever-present component of the energy-conserving inner membranes of bacteria and mitochondria. Its modulation of the structure and dynamism of the bilayer impacts on the activity of their resident proteins, as a number of studies have shown. Here we analyze the consequences cardiolipin has on the conformation, activity, and localization of the protein translocation machinery. C...