Results 31 to 40 of about 66,906 (291)

A phase 2/3 randomized clinical trial followed by an open-label extension to evaluate the effectiveness of elamipretide in Barth syndrome, a genetic disorder of mitochondrial cardiolipin metabolism

Genetics in Medicine, 2020
To evaluate effectiveness of elamipretide in Barth syndrome (BTHS), a genetic condition of defects in TAZ, which causes abnormal cardiolipin on the inner mitochondrial membrane.
W. Reid Thompson, B. Hornby, Ryan Manuel, E. Bradley, Jan-Jakob Laux, Jim Carr, H. Vernon   +6 more
semanticscholar   +1 more source

Thematic Review Series: Glycerolipids. Cardiolipin synthesis for the assembly of bacterial and mitochondrial membranes*

Journal of Lipid Research, 2008
In this article, the formation of prokaryotic and eukaryotic cardiolipin is reviewed in light of its biological function. I begin with a detailed account of the structure of cardiolipin, its stereochemistry, and the resulting physical properties, and I ...
Michael Schlame
doaj   +1 more source

Incorporation of cytochrome oxidase into cardiolipin bilayers and induction of nonlamellar phases. [PDF]

, 1990
Cytochrome oxidase from beef heart has been lipid-substituted with beef heart cardiolipin. The lipid phase behavior and protein aggregation state of the reconstituted complexes have been studied with 31P NMR, freeze-fracture electron microscopy, and ...
Knowles, P., Marsh, D., Powell, G.
core   +2 more sources

OPA1 disease alleles causing dominant optic atrophy have defects in cardiolipin-stimulated GTP hydrolysis and membrane tubulation [PDF]

, 2010
The dynamin-related GTPase OPA1 is mutated in autosomal dominant optic atrophy (DOA) (Kjer type), an inherited neuropathy of the retinal ganglion cells.
Alexander, Amati-Bonneau, Ardail, David C. Chan, Delettre, Delettre, Detmer, DeVay, Frezza, Griparic, Herlan, Heymann, Hudson, Ingerman, Jenny E. Hinshaw, Jürgen A.W. Heymann, Leonard, Meeusen, Olichon, Praefcke, Pucadyil, Rujiviphat, Sesaki, Song, Song, Tadato Ban, Tondera, Zhiyin Song, Zick   +28 more
core   +3 more sources

Hormone deprivation alters mitochondrial function and lipid profile in the hippocampus [PDF]

, 2017
Mitochondrial dysfunction is a common hallmark in aging. In the female, reproductive senescence is characterized by loss of ovarian hormones, many of whose neuroprotective effects converge upon mitochondria.
Alvarez, Silvia, Astiz, Mariana, Imsen, Mercedes, Magnani, Natalia Daniela, Merino, Florencia, Reines, Analia Gabriela, Seilicovich, Adriana, Zarate, Sandra Cristina   +7 more
core   +2 more sources

Biogenesis of the mitochondrial phosphate carrier [PDF]

, 1991
The mitochondrial phosphate carrier (PiC) is a member of the family of inner-membrane carrier proteins which are generally synthesized without a cleavable presequence.
Adrian G. S., Aquila H., Aquila H., Arends H., Chen W.-J., Cheng M. K., Daum G., Eilers M., Ferreira G. C., Freeman K. B., Goglia F., Kolbe H. V. J., Kramer R., Liu X., Melton D. A., Murakami K., Nicholson D. W., Pelham H. R. B., Pfanner N., Pfanner N., Pfanner N., Pfanner N., Pfanner N., Pfanner N., Pfanner N., Phelps A., Ricquier D., Ridley R. G., Runswick M. J., Runswick M. J., Schleyer M., Schleyer M., Schleyer M., Schwaiger M., Smagula C. S., Wachter E., Zimmermann R.   +36 more
core   +1 more source

Role of Cardiolipin in Mitochondrial Function and Dynamics in Health and Disease: Molecular and Pharmacological Aspects

Cells, 2019
In eukaryotic cells, mitochondria are involved in a large array of metabolic and bioenergetic processes that are vital for cell survival. Phospholipids are the main building blocks of mitochondrial membranes.
G. Paradies, Valeria Paradies, F. M. Ruggiero, G. Petrosillo   +3 more
semanticscholar   +1 more source

Effects of Copper and/or Cholesterol Overload on Mitochondrial Function in a Rat Model of Incipient Neurodegeneration [PDF]

, 2013
Copper (Cu) and cholesterol (Cho) are both associated with neurodegenerative illnesses in humans and animals models. We studied the effect in Wistar rats of oral supplementation with trace amounts of Cu (3 ppm) and/or Cho (2%) in drinking water for 2 ...
Arnal, Nathalie, Castillo, Hector Omar, Marra, Carlos Alberto, Tacconi, Maria Josefa   +3 more
core   +1 more source

Cytochrome c signalosome in mitochondria [PDF]

, 2011
Cytochrome c delicately tilts the balance between cell life (respiration) and cell death (apoptosis). Whereas cell life is governed by transient electron transfer interactions of cytochrome c inside the mitochondria, the cytoplasmic adducts of cytochrome
Díaz Moreno, Irene, Díaz Quintana, Antonio Jesús, García Heredia, José Manuel, Rosa Acosta, Miguel Ángel de la   +3 more
core   +1 more source

Arginine mutation alters binding of a human monoclonal antibody to antigens linked to systemic lupus erythematosus and the antiphospholipid syndrome [PDF]

, 2007
Objective: Previous studies have shown the importance of somatic mutations and arginine residues in the complementarity-determining regions (CDRs) of pathogenic anti-double-stranded DNA (anti-dsDNA) antibodies in human and murine lupus, and in studies of
Giles, I., Ioannou, Y., Isenberg, D.A., Lambrianides, A., Latchman, D.S., Manson, J.J., Mason, L., Rahman, A.   +7 more
core   +1 more source