Results 151 to 160 of about 2,620 (178)

Beyond Powerhouses: Roles of Mitochondria, from Development to Therapeutic Potential. [PDF]

Biology (Basel)
Pereira SP, Tavares LC.
europepmc   +1 more source

Contemporary insights into elamipretide's mitochondrial mechanism of action and therapeutic effects. [PDF]

Biomed Pharmacother
Sabbah HN, Alder NN, Sparagna GC, Bruce JE, Stauffer BL, Chao LH, Pitceathly RDS, Maack C, Marcinek DJ.   +8 more
europepmc   +1 more source

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 ...
Devrim Acehan, Zaza Khuchua, Riekelt H Houtkooper   +2 more
exaly   +5 more sources

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Cardiolipin remodeling and the function of tafazzin

Biochimica Et Biophysica Acta - Molecular and Cell Biology of Lipids, 2013
Cardiolipin, 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

Tafazzin splice variants and mutations in Barth syndrome

Molecular Genetics and Metabolism, 2014
Barth 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, Athena, Manolakos, Sarah Swain, Barnett, Iris L, Gonzalez   +3 more
openaire   +2 more sources

Cardiac mitochondrial structure and function in tafazzin-knockdown mice

Mitochondrion, 2018
Mutations 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, Kwangwon Lee, Hisashi Fujioka, Bernard Tandler, Charles L. Hoppel   +4 more
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A Reduction in Tafazzin Decreases Mitochondrial Function in C2C12 Myotubes

The FASEB Journal, 2022
Mitochondria, 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, Ashley N. Oliviera, David A. Hood   +2 more
openaire   +1 more source

Tafazzin knockdown causes hypertrophy of neonatal ventricular myocytes

American Journal of Physiology-Heart and Circulatory Physiology, 2010
Mutation of the mitochondrial protein tafazzin causes dilated cardiomyopathy in Barth syndrome. We employed an adenovirus as a vector to transfer tafazzin small hairpin RNA (shRNA) into neonatal ventricular myocytes (NVMs) to investigate the effects of tafazzin knockdown. The tafazzin shRNA adenovirus consistently knocked down tafazzin mRNA and lowered
openaire   +2 more sources

Drosophila Mitochondrial Membrane‐bound Tafazzin Protein Is A Transacylase

The FASEB Journal, 2007
Barth syndrome is an X‐linked mitochondria disorder with skeleton myopathy, cardiomyopathy and neutropenia, resulting from the tafazzin mutation. As demonstrated previously [Xu et al.: PNAS 103:11584, 2006], the mutation of full length Drosophila ...
Yang Xu, Mindong Ren, Ashim Malhotra, Louis Lee, Jin Zhang, Thomas JJ Blanck, Michael Schlame   +6 more
openaire   +1 more source