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TAZ (G4.5, BTHS, XAP-2, CMD3A, EFE, EFE2, Tafazzin, Barth syndrome, Protein G4.5, Endocardial fibroelastosis 2, Cardiomyopathy, dilated 3A (X-linked))

Cat no: 145249

TAZ (G4.5, BTHS, XAP-2, CMD3A, EFE, EFE2, Tafazzin, Barth syndrome, Protein G4.5, Endocardial fibroelastosis 2, Cardiomyopathy, dilated 3A (X-linked))

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.\n\nUniProt Number:\nQ16635\n\nGene ID:\nTAZ\n\nApplications:\nSuitable for use in Western Blot.\n\nRecommended Dilution:\nOptimal dilutions to be determined by the researcher.\n\nStorage and Handling:\nStore at -20?C for one year. After reconstitution, store at 4?C for one month. Can also be aliquoted and stored frozen at -20?C for long term.\nAvoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

145249

Size

100ug

Applications

WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

Supplied as a lyophilized powder. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

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.\n2. 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.\n3. 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.\n4. Hastings, R., Steward, C., Tsai-Goodman, B., Newbury-Ecob, R. Dysmorphology of Barth syndrome. Clin. Dysmorph. 18: 185-187, 2009.\n

Additional Info

Recognizes human,mouse and rat TAZ. No crossreactivity with other proteins.

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