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Omega-3 and omega-6 fatty acid differentially impact cardiolipin remodeling in activated macrophage

Autor: Wan-Hsin Chang, Hsiu-Chi Ting, Wei-Wei Chen, Jui-Fen Chan, Yuan-Hao Howard Hsu

Publikováno v: Lipids in Health and Disease, Vol 17, Iss 1, Pp 1-11 (2018)

Abstract Background The macrophage plays an important role in innate immunity to induce immune responses. Lipid replacement therapy has been shown to change the lipid compositions of mitochondria and potentially becomes an alternative to reduce the i

Externí odkaz: https://doaj.org/article/ca9790df0f944a0eb2cc3b960aee0c45

An improved functional assay in blood spot to diagnose Barth syndrome using the monolysocardiolipin/cardiolipin ratio

Autor: Femke S. Stet, Frédéric M. Vaz, Johanne H. Klinkspoor, Susan M. I. Goorden, Riekelt H. Houtkooper, Martin A. T. Vervaart, Hilary J. Vernon, Ronald J.A. Wanders, Henk van Lenthe, Willem Kulik

Publikováno v: Journal of inherited metabolic disease, 45(1), 29-37. Springer Netherlands

Barth syndrome is an X-linked disorder characterized by cardiomyopathy, skeletal myopathy and neutropenia, caused by deleterious variants in TAFAZZIN. This gene encodes a phospholipid-lysophospholipid transacylase that is required for the remodeling

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7d2bd8bd17b69fe1edd059a260cc8b45
https://doi.org/10.1002/jimd.12425

Diverse mitochondrial abnormalities in a new cellular model of TAFFAZZIN deficiency are remediated by cardiolipin-interacting small molecules

Autor: Arianna F. Anzmann, Olivia L. Sniezek, Steven M. Claypool, Alexandra Pado, Lauren R. DeVine, Hilary J. Vernon, Frédéric M. Vaz, Robert N. Cole, Anne Le, Simion Kreimer, Veronica F. Busa, Brian J. Kirsch

Publikováno v: Journal of biological chemistry, 297(3):101005. American Society for Biochemistry and Molecular Biology Inc.
The Journal of Biological Chemistry

Barth syndrome (BTHS) is an X-linked disorder of mitochondrial phospholipid metabolism caused by pathogenic variants in TAFFAZIN, which results in abnormal cardiolipin (CL) content in the inner mitochondrial membrane. To identify unappreciated pathwa

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8513153ad63be4ec2a55413a2dd75dea
https://pure.amc.nl/en/publications/diverse-mitochondrial-abnormalities-in-a-new-cellular-model-of-taffazzin-deficiency-are-remediated-by-cardiolipininteracting-small-molecules(0d7f8d5c-d63e-47b4-9cdd-35b501bb7f6b).html

Expression of human monolysocardiolipin acyltransferase-1 improves mitochondrial function in Barth syndrome lymphoblasts

Autor: Versha Banerji, Hana M. Zegallai, Grant M. Hatch, Edgard M. Mejia, Eric D.J. Bouchard, Amir Ravandi

Publikováno v: Journal of Biological Chemistry. 293:7564-7577

The mitochondrial polyglycerophospholipid cardiolipin (CL) is remodeled to obtain specific fatty acyl chains. This is predominantly accomplished by the transacylase enzyme tafazzin (TAZ). Barth syndrome (BTHS) patients with TAZ gene mutations exhibit

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::095105d8cac581abf5ac199800c3244d
https://doi.org/10.1074/jbc.ra117.001024