Catalogue number: | PA2135 |
Price: | $200.00 |
Reactivities: | Human, Mouse, Rat |
Applications: | Western Blot |
Size: | 100ug/vial |
Gene: | TAZ |
Swiss prot: | Q16635 |
Form: | Lyophilized |
Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing. |
Scientific background: | Tafazzin, also known as G4.5 is a protein that in humans is encoded by the TAZ gene. Cardiolipin is a complex glycerophospholipid with 4 acyl groups that localizes to the mitochondrial inner membrane and has a role in mitochondrial structure and function. By positional cloning, TAZ was identified within the critical Barth syndrome region on Xq28. Tafazzin is involved in the metabolism of cardiolipin. It is a component of the hippo signaling pathway thatcontrols tissue growth in animals. And it can function as aphospholipid lysophospholipid transacylase. |
References: | 1. Acehan, D., Vaz, F., Houtkooper, R. H., James, J., Moore, V., Tokunaga, C., Kulik, W., Wansapura, J., Toth, M. J., Strauss, A., Khuchua, Z. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. J. Biol. Chem. 286: 899-908, 2011. 2. Barth, P. G., Valianpour, F., Bowen, V. M., Lam, J., Duran, M., Vaz, F. M., Wanders, R. J. A. X-linked cardioskeletal myopathy and neutropenia (Barth syndrome): an update. Am. J. Med. Genet. 126A: 349-354, 2004. 3. Claypool, S. M., McCaffery, J. M., Koehler, C. M. Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins. J. Cell Biol. 174: 379-390, 2006. 4. Hastings, R., Steward, C., Tsai-Goodman, B., Newbury-Ecob, R. Dysmorphology of Barth syndrome. Clin. Dysmorph. 18: 185-187, 2009. |
Additional info: | A synthetic peptide corresponding to a sequence in the middle region of human TAZ, identical to the related mouse and rat sequences. |