Sodium 4-Phenylbutyrate-d11 is intended for use as an internal standard for the quantification of sodium 4-phenylbutyrate by GC- or LC-MS. Sodium 4-phenylbutyrate is a chemical chaperone that has been shown to rescue the trafficking of misfolded proteins.1,2 It also weakly blocks histone deacetylase activity (IC50 = 0.4 mM), which results in cell cycle arrest, differentiation, and/or apoptosis of various tumors.3,4 Formulations containing sodium 4-phenylbutyrate have been used for the treatment of urea cycle disorders.
1.Stewart, G.A., Ridscale, R., Martin, E.P., et al.4-Phenylbutyric acid treatment rescues trafficking and processing of a mutant surfactant protein-CAmerican Journal of Respiration, Cell, and Molecular Biology47(3)324-331(2012) 2.Bradbury, N.A.Focus on "sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of δF508-CFTR"Am. J. Physiol. Cell Physiol.278(2)C257-C258(2000) 3.Lu, Q., Wang, D.S., Chen, C.S., et al.Structure-based optimization of phenylbutyrate-derived histone deacetylase inhibitorsJ. Med. Chem.48(17)5530-5535(2005) 4.Ammerpohl, O., Trauzold, A., Schniewind, B., et al.Complementary effects of HDAC inhibitor 4-PB on gap junction communication and cellular export mechanisms support restoration of chemosensitivity of PDAC cellsBr. J. Cancer96(1)73-81(2007)