The cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome.

@article{Claypool2008TheCT,
  title={The cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome.},
  author={Steven M Claypool and Pinmanee Boontheung and J. Michael McCaffery and Joseph A Loo and Carla M Koehler},
  journal={Molecular biology of the cell},
  year={2008},
  volume={19 12},
  pages={
          5143-55
        }
}
Mutations in the mitochondrial cardiolipin (CL) transacylase, tafazzin (Taz1p), result in the X-linked cardioskeletal myopathy, Barth syndrome (BTHS). The mitochondria of BTHS patients exhibit variable respiratory defects and abnormal cristae ultrastructure. The biochemical basis for these observations is unknown. In the absence of its target phospholipid, CL, a very large Taz1p complex is missing, whereas several discrete smaller complexes are still observed. None of the identified Taz1p… CONTINUE READING