Results 161 to 170 of about 3,551 (191)
The Enzymatic Function of Tafazzin [PDF]
Journal of Biological Chemistry, 2006 Tafazzin is a putative enzyme that is involved in cardiolipin metabolism, it may carry mutations responsible for Barth syndrome. To identify the biochemical reaction catalyzed by tafazzin, we expressed the full-length isoform of Drosophila melanogaster tafazzin in a baculovirus-Sf9 insect cell system.
Michael Schlame
exaly +3 more sources
Some of the next articles are maybe not open access.
Related searches:
Related searches:
Cardiolipin remodeling and the function of tafazzin
Biochimica Et Biophysica Acta - Molecular and Cell Biology of Lipids, 2013Cardiolipin, the specific phospholipid of mitochondria, is involved in the biogenesis, the dynamics, and the supramolecular organization of mitochondrial membranes. Cardiolipin acquires a characteristic composition of fatty acids by post-synthetic remodeling, a process that is crucial for cardiolipin homeostasis and function.
Michael Schlame
exaly +3 more sources
The Function of Tafazzin, a Mitochondrial Phospholipid–Lysophospholipid Acyltransferase
Journal of Molecular Biology, 2020 Tafazzin is a mitochondrial enzyme that exchanges fatty acids between phospholipids by phospholipid-lysophospholipid transacylation. The reaction alters the molecular species composition and, as a result, the physical properties of lipids. In vivo, the most important substrate of tafazzin is the mitochondria-specific lipid cardiolipin.
Michael Schlame, Yang Xu
exaly +4 more sources
Barth syndrome mutations that cause tafazzin complex lability [PDF]
Journal of Cell Biology, 2011 Deficits in mitochondrial function result in many human diseases. The X-linked disease Barth syndrome (BTHS) is caused by mutations in the tafazzin gene TAZ1. Its product, Taz1p, participates in the metabolism of cardiolipin, the signature phospholipid of mitochondria.
Steven Claypool +2 more
exaly +5 more sources
Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria [PDF]
Mitochondrion, 2009 Tafazzin is a conserved mitochondrial protein that is required to maintain normal content and composition of cardiolipin. We used electron tomography to investigate the effect of tafazzin deletion on mitochondrial structure and found that cellular differentiation plays a crucial role in the manifestation of abnormalities. This conclusion was reached by
Zaza Khuchua +2 more
exaly +4 more sources
Tafazzin deficiency impairs CoA-dependent oxidative metabolism in cardiac mitochondria [PDF]
Journal of Biological Chemistry, 2020 Barth syndrome is a mitochondrial myopathy resulting from mutations in the tafazzin (TAZ) gene encoding a phospholipid transacylase required for cardiolipin remodeling. Cardiolipin is a phospholipid of the inner mitochondrial membrane essential for the function of numerous mitochondrial proteins and processes.
Jessica E Prenni +2 more
exaly +3 more sources
Tafazzin splice variants and mutations in Barth syndrome
Molecular Genetics and Metabolism, 2014Barth syndrome is caused by mutations in the TAZ (tafazzin) gene on human chromosome Xq28. The human tafazzin gene produces four major mRNA splice variants; two of which have been shown to be functional (TAZ lacking exon 5 and full-length) in complementation studies with yeast and Drosophila.
Susan M, Kirwin +3 more
openaire +2 more sources
Cardiac mitochondrial structure and function in tafazzin-knockdown mice
Mitochondrion, 2018Mutations in the tafazzin gene are the basis of Barth syndrome. The tafazzin protein is responsible for the synthesis of cardiolipin. Doxycycline-induced tafazzin-knockdown mice have been used as a model for Barth syndrome. In the current study, we examined subsarcolemmal and interfibrillar mitochondria from hearts of tafazzin-knockdown mice, focusing ...
Junhwan Kim +4 more
openaire +2 more sources
A Reduction in Tafazzin Decreases Mitochondrial Function in C2C12 Myotubes
The FASEB Journal, 2022Mitochondria, which are often regarded as the “powerhouse of the cell”, are labile organelles that regulate cellular metabolism, determine cell fate, and act as important signaling hubs. A phospholipid that is exclusively found within mitochondria that serves numerous roles is cardiolipin (CL).
Brandon J. Richards +2 more
openaire +1 more source