|Other Names||Trifunctional enzyme subunit beta, mitochondrial, TP-beta, 3-ketoacyl-CoA thiolase, Acetyl-CoA acyltransferase, Beta-ketothiolase, HADHB|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Cellular Location||Mitochondrion. Mitochondrion inner membrane Mitochondrion outer membrane Endoplasmic reticulum|
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Provided below are standard protocols that you may find useful for product applications.
HADHB encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.
Purevsuren, J., et al. Mol. Genet. Metab. 98(4):372-377(2009)Bogenhagen, D.F., et al. J. Biol. Chem. 283(6):3665-3675(2008)Wang, R., et al. Zhonghua Fu Chan Ke Za Zhi 41(10):672-675(2006)Hillier, L.W., et al. Nature 434(7034):724-731(2005)
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