Gene Symbol: TAZ1
Description: lysophosphatidylcholine acyltransferase
Alias: lysophosphatidylcholine acyltransferase
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Claypool S, Whited K, Srijumnong S, Han X, Koehler C. Barth syndrome mutations that cause tafazzin complex lability. J Cell Biol. 2011;192:447-62 pubmed publisher
    ..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...
  2. Osman C, Haag M, Wieland F, Brugger B, Langer T. A mitochondrial phosphatase required for cardiolipin biosynthesis: the PGP phosphatase Gep4. EMBO J. 2010;29:1976-87 pubmed publisher
    ..Our experiments provide the framework for the further dissection of mechanisms that are required for accumulation and maintenance of CL levels in mitochondria. ..
  3. Gebert N, Joshi A, Kutik S, Becker T, McKenzie M, Guan X, et al. Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome. Curr Biol. 2009;19:2133-9 pubmed publisher
    ..Our findings reveal a new role for cardiolipin in protein sorting at the mitochondrial outer membrane and bear implications for the pathogenesis of Barth syndrome. ..
  4. Claypool S, McCaffery J, Koehler C. Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins. J Cell Biol. 2006;174:379-90 pubmed
    ..Thus, BTHS can be caused by mutations that alter Taz1p sorting and assembly within the mitochondrion, indicating that the lipid target of Taz1p is resident to IMS-facing leaflets. ..
  5. Ma L, Vaz F, Gu Z, Wanders R, Greenberg M. The human TAZ gene complements mitochondrial dysfunction in the yeast taz1Delta mutant. Implications for Barth syndrome. J Biol Chem. 2004;279:44394-9 pubmed
    Barth syndrome is a genetic disorder that is caused by different mutations in the TAZ gene G4.5. The yeast gene TAZ1 is highly homologous to human TAZ, and the taz1Delta mutant has phospholipid defects similar to those observed in Barth ..
  6. Beranek A, Rechberger G, Knauer H, Wolinski H, Kohlwein S, Leber R. Identification of a cardiolipin-specific phospholipase encoded by the gene CLD1 (YGR110W) in yeast. J Biol Chem. 2009;284:11572-8 pubmed publisher
    ..We therefore rename the Ygr110wp as Cld1p (cardiolipin-specific deacylase 1). ..
  7. Chen S, He Q, Greenberg M. Loss of tafazzin in yeast leads to increased oxidative stress during respiratory growth. Mol Microbiol. 2008;68:1061-72 pubmed publisher
    The tafazzin (TAZ) gene is highly conserved from yeast to humans, and the yeast taz1 null mutant shows alterations in cardiolipin (CL) metabolism, mitochondrial dysfunction and stabilization of supercomplexes similar to those found in ..
  8. Brandner K, Mick D, Frazier A, Taylor R, Meisinger C, Rehling P. Taz1, an outer mitochondrial membrane protein, affects stability and assembly of inner membrane protein complexes: implications for Barth Syndrome. Mol Biol Cell. 2005;16:5202-14 pubmed
    The Saccharomyces cerevisiae Taz1 protein is the orthologue of human Tafazzin, a protein that when inactive causes Barth Syndrome (BTHS), a severe inherited X-linked disease...
  9. Testet E, Laroche Traineau J, Noubhani A, Coulon D, Bunoust O, Camougrand N, et al. Ypr140wp, 'the yeast tafazzin', displays a mitochondrial lysophosphatidylcholine (lyso-PC) acyltransferase activity related to triacylglycerol and mitochondrial lipid synthesis. Biochem J. 2005;387:617-26 pubmed
    ..The relationship between the deletion of the YPR140w gene and the lipid composition of the ypr140wDelta cells is discussed. ..

More Information


  1. Gu Z, Valianpour F, Chen S, Vaz F, Hakkaart G, Wanders R, et al. Aberrant cardiolipin metabolism in the yeast taz1 mutant: a model for Barth syndrome. Mol Microbiol. 2004;51:149-58 pubmed
    ..5 (also known as tafazzin). We constructed a yeast mutant (taz1) containing a null mutation in the homologue of the human G4.5 gene...
  2. Rijken P, Houtkooper R, Akbari H, Brouwers J, Koorengevel M, de Kruijff B, et al. Cardiolipin molecular species with shorter acyl chains accumulate in Saccharomyces cerevisiae mutants lacking the acyl coenzyme A-binding protein Acb1p: new insights into acyl chain remodeling of cardiolipin. J Biol Chem. 2009;284:27609-19 pubmed publisher
    ..Based on the findings, we conclude that the fatty acid composition of mature CL in yeast is governed by the substrate specificity of the CL-specific lipase Cld1p and the fatty acid composition of the Taz1p substrates. ..
  3. Baile M, Whited K, Claypool S. Deacylation on the matrix side of the mitochondrial inner membrane regulates cardiolipin remodeling. Mol Biol Cell. 2013;24:2008-20 pubmed publisher
    ..Mutations in TAZ1 result in the X-linked cardiomyopathy Barth syndrome...
  4. Ye C, Lou W, Li Y, Chatzispyrou I, Huttemann M, Lee I, et al. Deletion of the cardiolipin-specific phospholipase Cld1 rescues growth and life span defects in the tafazzin mutant: implications for Barth syndrome. J Biol Chem. 2014;289:3114-25 pubmed publisher
    ..We show that cld1? rescues growth, life span, and respiratory defects of the taz1? mutant...
  5. Gaspard G, McMaster C. The mitochondrial quality control protein Yme1 is necessary to prevent defective mitophagy in a yeast model of Barth syndrome. J Biol Chem. 2015;290:9284-98 pubmed publisher
    The Saccharomyces cerevisiae TAZ1 gene is an orthologue of human TAZ; both encode the protein tafazzin...
  6. Tyurina Y, Lou W, Qu F, Tyurin V, Mohammadyani D, Liu J, et al. Lipidomics Characterization of Biosynthetic and Remodeling Pathways of Cardiolipins in Genetically and Nutritionally Manipulated Yeast Cells. ACS Chem Biol. 2017;12:265-281 pubmed publisher
    ..differential phospholipidomics analysis, we evaluated the contribution of Cld1 (CL-specific phospholipase A) and Taz1 (acyl-transferase) as the major regulatory mechanisms of the remodeling process...
  7. Herndon J, Claypool S, Koehler C. The Taz1p transacylase is imported and sorted into the outer mitochondrial membrane via a membrane anchor domain. Eukaryot Cell. 2013;12:1600-8 pubmed publisher
    ..These studies suggest that altering the mitochondrial import pathway of Taz1p may be important in understanding the molecular basis of Barth syndrome. ..
  8. Abe M, Hasegawa Y, Oku M, Sawada Y, Tanaka E, Sakai Y, et al. Mechanism for Remodeling of the Acyl Chain Composition of Cardiolipin Catalyzed by Saccharomyces cerevisiae Tafazzin. J Biol Chem. 2016;291:15491-502 pubmed publisher
    ..The acyl specificity of tafazzin may be one of the factors that determine the acyl composition of mature CL in S. cerevisiae mitochondria. ..