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(Sodium) PHENYLBUTYRATE (Sodium) PHENYLACETATE

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CANCER RESEARCH

Brain

Samid D., Wells M., Greene ME, Shen, W., Palmer CN, Thibault, A. Peroxisome Proliferator-activated Receptor Gamma as a Novel Target in Cancer Therapy: Binding and Activation by an Aromatic Fatty Acid with Clinical Antitumor Activity. Clin Cancer Res. 6(3):933-41. Mar 2000.

Ozawa T., Lu R.M., Hu L.J., Lamborn K.R., Prados M.D., and Deen D.F. Radiopetentiation of human brain tumor cells by sodium Phenylacetate. Elsevier, Cancer Letters, 142 (1999):139-142., Mar 1999.

Chang SM, Kuhn JG, Robins HI, Schold SC, Spence AM, Berger MS, Mehta MP, Bozik ME, Pollack I, Gilbert M, Rankin C, Prados MD. Phase II study of Phenylacetate in patients with recurrent malignant glimoa: a North American Brain Tumor Consortium report. J Clin Oncol., 17:984-990, Mar 1999.

Pelidis M.A., Carducci M.A., Simons J.W. Cytotoxic Effects of Phenylbutyrate on Human Neuroblastoma Cell Lines. Int. J. Oncol., 12 (4): 889-93. Apr 1998.

Englehard H., Homer R.J., Duncan H., Rozental J. Inhibitory Effects of Phenylbutyrate on the Proliferation, Morphology, Migration and Invasiveness of Malignant Glioma Cells. J. Neuro-Oncol., 37:97-108, Apr 1998.

Lau CC., Parikh S., Li X.N., Chow C., Jung H.L., Blaney S. Differentiation Induction in Medulloblastoma Cell Lines by Phenylbutyrate. Proceedings of the AACR, Mar 1998.

Sidnell N, Chang B, Yamashiro JM, Wada RK. Transcriptional up-regulation of retinoic acid receptor b (RAR b) expression by Phenylacetate in human neuroblastoma cells. Exp. Cell Res., 239:169-174, 1998.

Pelidis MA, Carducci MA, Simons JW. Integration of sodium phenylbutyrate into neuroblastoma therapy. Int J Oncol, 12(4):889-93. 1998.

Engelhard H., Duncan H., Rozental J. Phenylbutyrate Down-modulates Glioblastoma Cell Invasiveness and the Expression of Phosphorylated Retinoblastoma Protein, c-myc and Urokinase. Proceedings of the AACR, Mar 1997.

Liu L., Kulkarni M., Thibault A., Samid D. Down-regulation of Cyclin D1 Expression in Human Glioma Cells by the Differentiation Inducers Phenylacetate and Phenylbutyrate. Proceedings of the AACR, Mar 1997.

Serabe B., Adamson P., Wolfe R., Balis F., et al. Phase I Trial and Pharmacokinetic (PK) Study of Phenylacetate (PA) Given as a 28-day Continuous Infusion (CIVI) in Children. Proceedings of the AACR, Mar 1997.

Wada RK, Huang J., Yamashiro J., Shimoda L., Sidell N. Combination Therapy with Retinoic Acid and Phenylacetate Induces Differentiation and N-myc Down-regulation in a Resistant Neuroblastoma Cell Line. Proceedings of the AACR, Mar 1997.

Lu RM, Ozawa T, Hu LJ, Prados MD, Deen DF. In vitro cytotoxicity and radiopotentiation of Phenylacetate in human brain tumor cells. Proceedings of the AACR., Vol. 38, Mar 1997.

Miller AC, Whittaker A, Thibault A, Samid D. Modulation of radiation response of human tumor cells by the differentiation inducers, Phenylacetate. Int J Radiat Biol, 72:211-218. 1997.

Boudoulas S., Lush RM., McCall N.A., Samid D., Reed E., Figg WD. Plasma Protein Binding of Phenylacetate and Phenylbutyrate, Two Novel Antineoplastic Agents. Ther Drug Monit., 18:714-720. Dec 1996.

Pelidis MA, Carducci MA, Simons JW. Integration of sodium phenylbutyrate into neuroblastoma therapy. Proceedings of ASCO, Vol. 15A. May 1996.

Pineau T., Hudgins W., Liu L., Chen L., Sher T., Gonzales F., Samid D. Activation of a Human Peroxisome Proliferator-Activated Receptor by the Antitumor Agent Phenylacetate and its Analogs. Biochemical Pharmacol., 56:659-667. 1996.

Prasanna P., Thibault A., Liu L., Samid D. Lipid Metabolism as a Target for Brain Cancer Therapy: Synergistic Activity of Lovastatin and Sodium Phenylacetate Against Human Glioma Cells. J. Neurochem,. 66: 710-716. 1996.

Prasanna P., Shack S., Wilson V., Samid D. Phenylacetate in Chemoprevention: in vitro and in vivo Suppression of 5-Aza-2'-Deoxycytidine-induced Carcinogenesis. Clin Cancer Res., 1:865-871, Aug 1995.

Liu L, Shack S, Stetler-Stevenson WG, Hudgins WR, Samid D. Mutant p53 as a Target of Phenylacetate in human glioblastoma. Proceedings of the AACR, Mar 1995.

Miller A, Shack S, Samid D. Phenylacetate-induced modulation of radiation response in prostate carcinoma, breast adenocarcinoma and glioblastoma cell lines: time factor and mechanistic. Proceedings of the AACR, Mar 1995.

Thibault A, Samid D, Cooper M, Figg W, et al. Phase I Study of Phenylacetate Administered Twice Daily to Patients with Cancer. Cancer, 75:2932-2938, Feb 1995.

Hudgins W., Shack S., Myers C., Samid D. Cytostatic Activity of Phenylacetate and Derivatives Against Tumor Cells: Correlation with Lipophilicity and Inhibition of Protein Prenylation. Biochemical Pharmacol., 50:1273:1279. 1995.

Sidell N., Wada R., Han G., Chang B., Shack S., Moore T., Samid D. Phenylacetate Synergizes with Retinoic Acid in Inducing Differentiation of Human Neuroblastoma Cells. Int. J. Cancer., 60: 507-14. 1995.

Stockhammer G., Manley, G. T., Johnson R., Rosenblum M., Samid D., Lieberman F. Inhibition of Proliferation and Induction of Differentiation in Medulloblastoma and Astrocytoma-derived Cell Lines with Phenylacetate. J. Neurosurgery., 83:672-81. 1995.

Lui L, Bar-Ner M, Weber J, Danielpour D, Quain S, Shearer G, Samid D. Enhancement of tumor immuno-genicity by Phenylacetate and derivatives: changes in surface antigens and tumor-derived immunosuppressive factors. Proceeding of the AACR, Apr 1994.

Samid D, Hudgins WR, et al. Phenylacetate and derivatives: simple compounds with complex antitumor activities. Proceedings of the AACR, Apr 1994.

Thibault A., Cooper M., Figg W., Venzon D. A Phase I and Pharmacokinetic Study of Intravenous Phenylacetate in Patients with Cancer. Cancer Res., 54:1690-94. Apr 1994.

Samid D., Ram Z., Hudgins W.R., Shack S., Liu, L., Walbridge S., Oldfield E., Myers C. Selective Activity of Phenylacetate Against Malignant Gliomas: Resemblance to Fetal Brain Damage in Phenylketonuria. Cancer Res., 54:891-95, Feb 1994.

Ram Z., Samid D., Walbridge S., Oshiro E., et al. Growth Inhibition, Tumor Maturation and Extended Survival in Experimental Brain Tumors in Rats Treated with Phenylacetate. Cancer Res,. 54:2923-27. 1994.

Cinatl Ji., Cinatl Ja, Mainke M., et al. In vitro Differentiation of Human Neuroblastoma Cells Induced by Sodium Phenylacetate. Cancer Letters., 70: 15-24. 1993.

Wada R, Han G, Moore T, Samid D, Sidell N. Effects of Phenylacetate and its interaction with reinoic acid on human neuroblastoma differentiation. Proceedings of the AACR, Apr 1990.

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Breast

Samid D., Wells M., Greene ME, Shen, W., Palmer CN, Thibault, A. Peroxisome Proliferator-activated Receptor Gamma as a Novel Target in Cancer Therapy: Binding and Activation by an Aromatic Fatty Acid with Clinical Antitumor Activity. Clin Cancer Res. 6(3):933-41. Mar 2000.

Thibout, D., Kraemer, M., Di Benedetto, et al. Sodium Phenylacetate (NaPa) Induces Modifications of the Proliferation, the Adhesion and the Cell Cycle of Tumoral Epithelial Breast Cells. Anti Cancer Research. 19(3A):2121-6. May-Jun 1999.

Carducci M., Bowling MK., Eisenberger M., Sinibaldi V., Chen T.L., Noe D., Grochow L., Donehower R. Phenylbutyrate (PB) for Refractory Solid Tumors: Phase I Evaluation of Continuous Oral PB Exposure. Proceedings of the AACR, Mar 1998.

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

Adam L., Crépin M., Israël L. Tumor Growth Inhibition, Apoptosis, and Bcl-2 Down-regulation of MCF-7ras Tumors by Sodium Phenylacetate and Tamoxifen Combination. Cancer Res., 57:1023-1029. Mar 1997.

Miller AC, Whittaker A, Thibault A, Samid D. Modulation of radiation response of human tumor cells by the differentiation inducers, Phenylacetate. Int J Radiat Biol, 72:211-218. 1997.

Gorospe M., Shack S. Guyton K.Z., Samid D., Holbrook N.J. Up-regulation and Functional Role of p21Waf1/Cip1 During Growth Arrest of Human Breast Carcinoma MCF-7 Cells by Phenylacetate. Cell Growth Differ., 7(12): 1609-1615. Dec 1996.

Shack S., Miller A., Liu L., Thibault A., Prasanna P., Samid D. Vulnerability of Multidrug-resistant Tumor Cells to the Aromatic Fatty Acids Phenylacetate and Phenylbutyrate. Clin. Cancer Res., 2:865-872. May 1996.

Johnson DE, Ochieng J, Evans SL. Phenylacetic acid halides inhibit estrogen receptor (ER)-postive MCF-7 cells, but not ER-negative breast cancer cells or normal breast epithelial cells. Anticancer Drug, 7:288-292. 1996.

Adam L., Crépin M., Savin C., Israël L. Sodium Phenylacetate Induces Growth Inhibition and Bcl-2 Down-regulation and Apoptosis in MCF7ras Cells in Vitro and in Nude Mice. Cancer Res., 55: 5156-5160. Nov 1995.

Miller A, Shack S, Samid D. Phenylacetate-induced modulation of radiation response in prostate carcinoma, breast adenocarcinoma and glioblastoma cell lines: time factor and mechanistic. Proceedings of the AACR, Mar 1995.

Shack S, Liu L, Miller A, Thibault A, Prasanna P, Samid D. Differentiation therapy using the aromatic fatty acids Phenylacetate and phenylbutyrate as an alternative treatment for multiple drug resistant tumors: in vitro studies. Proceedings of the AACR, Mar 1995.

Samid D, Hudgins WR, et al. Phenylacetate and derivatives: simple compounds with complex antitumor activities. Proceedings of the AACR, Apr 1994.

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Colon

Huang Y., Horvath CM, Waxman S., Regrowth of 5-Fluorouracil-treated Human Colon Cancer Cells is Prevented by the Combination of Interferon Gamma, Indomethacin, and Phenylbutyrate. Cancer Res. 60(12):3200-6. Jun 15, 2000.

Sung, M.W., and Waxman, S. Chemodifferentiation Therapy with Fluorouracil (FU) and Phenyl-butyrate (PB) in Advanced Colorectal Cancer: A Phase I Trial. Proceedings of the AACR, Apr 1999.

Huang Y., Waxman S. Enhanced Growth Inhibition and Differentiation of Fluorodeoxyuridine-treated Human Colon Carcinoma Cells by Phenylbutyrate. Clin Cancer Res.,4 (10): 2503-9. Oct 1998.

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

Huang Y., Waxman S. Enhanced p21WAF1 Expression, Differentiation, and Growth Inhibition of Human Colon Carcinoma Cell Lines Result from Combined Treatment with Interferon g (IFNg) and Phenylbutyrate (PB). Proceedings of the AACR, Mar 1997.

Miller AC, Whittaker A, Thibault A, Samid D. Modulation of radiation response of human tumor cells by the differentiation inducers, Phenylacetate. Int J Radiat Biol, 72:211-218. 1997.

Shack S., Miller A., Liu L., Thibault A., Prasanna P., Samid D. Vulnerability of Multidrug-resistant Tumor Cells to the Aromatic Fatty Acids Phenylacetate and Phenylbutyrate. Clin. Cancer Res., 2:865-872. May 1996.

Shack S, Liu L, Miller A, Thibault A, Prasanna P, Samid D. Differentiation therapy using the aromatic fatty acids Phenylacetate and phenylbutyrate as an alternative treatment for multiple drug resistant tumors: in vitro studies. Proceedings of the AACR, Mar 1995.

Samid D, Hudgins WR, et al. Phenylacetate and derivatives: simple compounds with complex antitumor activities. Proceedings of the AACR, Apr 1994.

Clarke K., Feinman R., Harrison LE. Phenylbutyrate-Induced Apoptosis is Associated with Inactivation of Nf-кB. Proceedings of the AACR. Vol. 42, March 2001.

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Hodgkin's

Bar-Ner M, Thibault A, Tsokos M, Magrath IT, Samid D. Phenylbutyrate induces cell differentiation and modulates Espein-Barr virus gene expression in Burkitt's lymphoma cells. Clin Cancer Res., 5:1509-1516, 1999.

Boudoulas S., Lush RM., McCall N.A., Samid D., Reed E., Figg WD. Plasma Protein Binding of Phenylacetate and Phenylbutyrate, Two Novel Antineoplastic Agents. Ther Drug Monit., 18:714-720. Dec 1996.

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Leukemia

Gore SD, Carducci MA. Modifying histones to tame cancer: cliical development of sodium phenylbutyrate and other histone deacetylace inhibitors. Expert Opin Investig Drug, (12):2923-34. Dec 2000.

Maslak P., Scheinberg D., Targeted therapies for the myeloid leukaemias. Expert Opin Investig Drugs. 9(6):1197-205 Jun 2000.

Chung YL., Lee YH., Yen SH., Chi KH., A novel approach for nasopharyngeal carcinoma treatment uses phenylbutyrate as a protein kinase C modulator: implications for radiosensitization and EBV-targeted therapy. Clin Cancer Res. 6(4):1452-8. April 2000.

Witzig T.E., Timm M., Stenson M., Kaufmann S.H. Induction of Apoptosis in Malignant B Cells by Phenylbutyrate or Phenylacetate in Combination with Chemotherapeutic Agents. Clinical Cancer Research 6(2): 681-92. Feb 2000.

DiGiuseppe JA, Weng LJ, Yu KH, Fu S, Kastan MB, Samid, D., Gore, SD. Phenylbutyrate-induced G1 Arrest and Apoptosis in Myeloid Leukemia Cells: Structure-Function Analysis. Leukemia 13(8):1243-53. Aug 1999.

Yu K.H., Weng L.J., Gore S.D. Augmentation of Phenylbutyrate -induced Differentiation of Myeloid Leukemia Cells Using all-trans Retinoic Acid. Leukemia 13(8):1258-65. Aug 1999.

Lea M.A., Hodge S.K., and Randolph V.M. Growth regulation and induction of histone acetylation by 4-phenylbutyrate and structural analogs. Proceedings of the AACR. Vol. 40, Apr 1999.

Lea M.A., Hodge, S. K., Randolph V.M. Induction of Histone Acetylation and Growth Regulation in Eryrthroleukemia Cells by 4-Phenylbutyrate and Structural Analogs. UMDNJ - New Jersey Medical School, Newark, NJ. Anticancer Res., 19(3A):1971-6. 1999.

Warrell R.P., He L.Z., Richon V, Calleja E., Pandolif P.P., Therapeutic Targeting of Transcription in Acute Promyelocytic Leukemia by Use of Inhibitor of Histone Deacetylase. J Natl Cancer Inst., 490(21):1621-5. Nov 1998.

List A.F., Hematopoietic Stimulation by Amifostine and Sodium Phenylbutyrate: What is the Potential in MDS? Leuk Res., Suppl 22:S7-11. May 1998.

Fu S., Edenfield J., Weng LJ, Gore SD. Synergistic Induction of Apoptosis in Myeloid Leukemia Cells by Combination of two Putative Differentiation Inducers, Vesnarinone and Sodium Phenylbutyrate. Proceedings of the AACR, Mar 1998.

Gore Steven D., Dvorit Samid, and Li-Jun Weng Impact of the Putative Differentiating Agents Sodium Phenylbutyrate and Sodium Phenylacetate on Proliferation, Differentiation, and Apoptosis of Primary Neoplastic Myeloid Cells. Clin Cancer Res., 3:1755-1762. Oct 1997.

Rivero J.A., Adunyah SE, Ceesay KJ. Phenylbutyrate, Sodium Butyrate and Phenylacetate Affect of the Levels of p34cdc2 kinase and PKC in K562 Erythroleukemic Cells. Proceedings of the AACR, Mar 1996.

Weng LJ, Burke PJ, Gore SD. Sodium Phenylbutyrate: Potential Differentiating Agent for Myeloid Malignancies. Proceedings of the AACR, Mar 1996.

Lea MA, Tulsyan N. Discordant effects of butyrate analogues on erythroleukemia cell proliferation, differentiation and histone deacetylase. Anticancer Res., 15:879-883, 1995.

Lui L, Bar-Ner M, Weber J, Danielpour D, Quain S, Shearer G, Samid D. Enhancement of tumor immuno-genicity by Phenylacetate and derivatives: changes in surface antigens and tumor-derived immunosuppressive factors. Proceeding of the AACR, Apr 1994.

Call CDRT, Stenson MJ, Witzig TE. Effects of Phenylacetate on cells from patients with B-chronic lymphocytic leukemia. Luk Lymphoma., 14:145-149, 1994.

Samid D., Yeh A., Prasanna P. Induction of Erythroid Differentiation and Fetal Hemoglobin Production in Human Leukemic Cells Treated with Phenylacetate. Blood. 80: 1576-1581, Sep 1992.

Samid D, Shack S, Myers CE. Phenylacetate in suppression of human prostate adenocarcinoma cell growth and invasion. Proceedings of the AACR, May 1992.

Samid D, Shack S, Myers CE, Prasanna P. Phenyl-acetate, a novel inducer of tumor cell differentiation. Proceedings of the AACR, May 1992.

Samid D, Shack S, Sherman LT. Phenylacetate: A novel nontoxic inducer of tumor cell differentiation. Cancer Res. 1988-1992. Apr 1992.

Samid D, Shack S, Yeh TJ, Sherman LT. Induction of tumor cell differentiation by nontoxic phenyl derivatives. Proceedings of the AACR, May 1991.

Samid D, Yeh TJ, Shack S. Interferon in combination with antitumourigenic phenyl derivatives: potentiation of IFNa activity in-vitro. BR J Haematol; 79(suppl 1):81-83. 1991.

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Prostate

Reynolds S., Cederberg H., Chakrabarty S. Inhibitory effect 1-0 (2 methoxy) hexadecyl glycerol and Phenylbutyrate o the malignant properties of human prostate cancer cells. Clinical & Experimental Metastasis. 18: 309-312. Dec 2000.

Ng AY, Bales W, Veltri RW. Phenylbutyrate-induced apoptosis and differential expression of Bcl-2, Bax, p53 and Fas in human prostate cancer cell lins. Analytical and quantitative Cytology and Histology, 45-54. Jun 1999.

Veltri RW, Bales WD, Brights S, Vesella RA, Ng AY. Effect of phenylbutyrate on in expression of prostate associated tumor biomakers. Proceedings of the AACR., Vol. 40, Apr 1999.

Ludeman, S.M., Carducci, M.A. Springer, et al. Analogs of Phenylbutyrate as Mechanistic Probes of Differentiation. Proceedings of the AACR, Apr 1999.

Melchior SW, Brown LG, Figg WD, Quinn JE, Santucci RA, Brunner J, Thuroff JW, Lange PH, Vessella RL. Effect of phenylbutyrate on proliferation and apoptosis in human prostate cancer cells in vitro and in vivo. Int. J. Oncol., 14:501-508, 1999.

Gao M, Ossowski L, Ferrari AC. Molecular mechanisms of phenylbutyrate induced apoptosis in prostate cancer cells. Proceedings of the AACR., Vol. 39, Mar 1998.

Yang Q, Tong K, David-Beabes G, Meeker A, Carducci MA. Phenylbutyrate (PB) induces cyclin-dependent kinase regulators, suppresses toposiomerase 11a, and inhibits telomerase activity in human prostate cancer (PCA). Proceedings of the AACR., Vol. 39, Mar 1998.

Carducci M., Bowling MK., Eisenberger M., Sinibaldi V., Chen T.L., Noe D., Grochow L., Donehower R. Phenylbutyrate (PB) for Refractory Solid Tumors: Phase I Evaluation of Continuous Oral PB Exposure. Proceedings of the AACR, Mar 1998.

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

Hamilton G, Haberl I, Jager W, et al. Phenylacetate and sodium selenite in vitro increase the cellular damage induced in PC3 prostate cancer cells by pretreatment with campthotecines. Proceedings of the AACR, 39:527. 1998.

Tong KP, David-Beabes G, Meeker A, Bucci J, DeWeese T, Carducci MA. Phenylbutyrate (PB) has pleiotropic effects on gene transcription and inhibits telomerase activity in human prostate cancer. Conference on Differentiation Therapy, Oct 1997.

Melchoir S, Brown L, Quinn J, Santucci R, Lange PH, Vesella RA. Effects of phenylbutyrate on cell and apoptosis in human prostate cancer cells. Proceedings the AACR., Vol. 38, Mar 1997.

Carducci MA, Bowling MK, Eisenberger M, Sinibaldi V, Chen T, Noe D, Growhow L, Donehower R. Phenylbutyrate (PB) for refractory solid tumors: Phase I clinical and pharmacological evaluation of intravenous and oral PB. Anticancer Res., 17:3921-3982, 1997.

Miller AC, Whittaker A, Thibault A, Samid D. Modulation of radiation response of human tumor cells by the differentiation inducers, Phenylacetate. Int J Radiat Biol, 72:211-218. 1997.

Boudoulas S., Lush RM., McCall N.A., Samid D., Reed E., Figg WD. Plasma Protein Binding of Phenylacetate and Phenylbutyrate, Two Novel Antineoplastic Agents. Ther Drug Monit., 18:714-720. Dec 1996.

Carducci M., Bowling M., Eisenberger M., Sinibaldi V., Simons J., Chen T., Noe D., Grochow L., Donehower R. Phenylbutyrate (PB) for Refractory Solid Tumors: A Phase I Clinical and Pharmacological Evaluation. Proceedings of the AACR, Mar 1996.

Melchoir S, Stone B, Santucci R, Brown L, True L, Daniel J, Lange P, Vesella RA. Differentiation induces Phenylacetate and phenylbutyrate and their effects in vitro and on the advanced prostate cancer (CaP) xenograft model LuCaP 23.1. Proceedings of the AACR., Vol. 37, Mar 1996.

Tong KP, DeWeese TL, Mansfield EP, Carducci MA. New insights into phenylbutyrate bioactivity in human prostate cancer. Proceedings of the AACR., Vol. 37, Mar 1996.

Danesi R, Nardini D, Basolo F, Del Tacca M, Samid D, Myers CE. Phenylacetate inhibits isoprenylation and growth of the androgen-independent LNCaP prostate cancer cells transfected with the T24 Ha-ras oncogene. Mol. Pharmacol., 49:972-979, Mar 1996.

Carducci MA, Nelson J, Chan-Tack K, Ayyagari S, Sweatt W, Campbell P, Nelson W, Simons J. Phenylbutyrate induces apoptosis in human prostate cancer and is more potent than Phenylacetate. Clin Cancer Res., 2:379-387, Feb 1996.

Pineau T., Hudgins W., Liu L., Chen L., Sher T., Gonzales F., Samid D. Activation of a Human Peroxisome Proliferator-Activated Receptor by the Antitumor Agent Phenylacetate and its Analogs. Biochemical Pharmacol., 56:659-667. 1996.

Bowling MK, Nelson JB, Tong KP, Simons JW, Eisenberger MA, Sinibaldi V, Donehower RC, Carducci MA. Biomarker responses in men with advanced prostate cancer (PCA) to infusional phenylbutyrate (PB). Proc. Am. Soc. Clin. Oncol., 15:A43, 1996.

Walls R, Thibault A, Lui L, Wood C, Kozlowski JM, Figg WD, Sampson ML, Elin RJ, Samid D. The differentiating agent Phenylacetate increases prostate-specific antigen production by prostate cancer cells. The Prostate; 29:177-182. 1996.

Ayyagari S, Sweatt W, Campbell P, Nelson W, Simins J, Carducci MA. Phenylbutyrate, a novel compound for human prostate cancer therapy, induces apoptosis and has cytotoxic effects more potent than Phenylacetate. Clin Cancer Res, 2(2):379-87. 1996.

Hamilton G, Haberl I, Theyer G, Baumgartner G, Wenzl E. In vitro sensitivity of Phenylacetate-induced cancer-derived cell lines to anthracyclines and camphothecines. Proceedings of the AACR, Mar 1995.

Miller A, Shack S, Samid D. Phenylacetate-induced modulation of radiation response in prostate carcinoma, breast adenocarcinoma and glioblastoma cell lines: time factor and mechanistic. Proceedings of the AACR, Mar 1995.

Wood C, Sensibar J, Lee C, Kozlowski J. Phenylacetate and phenylbutyrate promote cellular differentiation through downregulation of androgen receptor and bci-2 levels in the prostate cancer cell line LNCaP. Proceedings for AACR, 36:A3831, Mar 1995.

Thibault A, Samid D, Cooper M, Figg W, et al. Phase I Study of Phenylacetate Administered Twice Daily to Patients with Cancer. Cancer, 75:2932-2938, Feb 1995.

Carducci MA, Ayyagari S, Sweatt W, Campbell P, Nelson W, Simons J. Phenylbutyrate, a novel compound for human prostate cancer, induces apoptosis and has cytotoxic effects more potent that Phenylacetate. Proceedings of the AACR, 36:392, 1995.

Hudgins W., Shack S., Myers C., Samid D. Cytostatic Activity of Phenylacetate and Derivatives Against Tumor Cells: Correlation with Lipophilicity and Inhibition of Protein Prenylation. Biochemical Pharmacol., 50:1273:1279. 1995.

Samid D, Hudgins WR, et al. Phenylacetate and derivatives: simple compounds with complex antitumor activities. Proceedings of the AACR, Apr 1994.

Thibault A., Cooper M., Figg W., Venzon D. A Phase I and Pharmacokinetic Study of Intravenous Phenylacetate in Patients with Cancer. Cancer Res., 54:1690-94. Apr 1994.

Figg W, Walls R, Cooper M, Thibault A, Sartor O, McCall N, Myers C, Samid D. In vitro antitumor effect of hydroxyurea on hormone-refractory prostate cancer cells and its potentiation by phenylbutyrate. Anticancer Drugs, 5:336-342, 1994.

Samid D, Shack S, Myers CE. Selective growth arrests and phenotypic reversion of prostate cancer cells in vitro by nontoxic pharmacological concentration of Phenylacetate. J. Clin. Invest., 91:2288-2295, May 1993.

Wood CG, Sensiber J, Lee C, Kozowski JM. Phenylacetate and phenylbutyrate promote cellular differentiation in human prostate cancer systems. Proceedings of the AACR, May 1991.

Samid D, Yeh TJ, Shack S. Interferon in combination with antitumourigenic phenyl derivatives: potentiation of IFNa activity in-vitro. BR J Haematol; 79(suppl 1):81-83. 1991.

Pili R., Lantz J., Kruszewski MP., Weeraratna A., Carducci MA. Molecular Mechanisms for the Antitumor Effect of Phenylbutyrate and 13-Cis Retinoic Acid, and Sequence-dependent Effect in Combination with Paclitaxel. Proceedings of the AACR. Vol. 42, March 2001.

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Skin

Boudoulas S., Lush RM., McCall N.A., Samid D., Reed E., Figg WD. Plasma Protein Binding of Phenylacetate and Phenylbutyrate, Two Novel Antineoplastic Agents. Ther Drug Monit., 18:714-720. Dec 1996.

Liu L., Hudgins R., Miller A., Samid D. et al Transcriptional Upregulation of TGF-a by Phenylacetate and Phenylbutyrate is Associated with Differentiation of Human Melanoma Cells. Cytokine., 7:449-456, Jul 1995.

Hudgins W., Shack S., Myers C., Samid D. Cytostatic Activity of Phenylacetate and Derivatives Against Tumor Cells: Correlation with Lipophilicity and Inhibition of Protein Prenylation. Biochemical Pharmacol., 50:1273:1279. 1995.

Samid D, Hudgins WR, et al. Phenylacetate and derivatives: simple compounds with complex antitumor activities. Proceedings of the AACR, Apr 1994.

Liu L., Shack S., Stetler-Stevenson W., Hudgins W., Samid D. Differentiation of Cultured Human Melanoma Cells Induced by the Aromatic Fatty Acids Phenylacetate and Phenylbutyrate. J. Invest. Dermatol., 103:335-340. 1994.

Samid D, Yeh TJ, Shack S. Interferon in combination with antitumourigenic phenyl derivatives: potentiation of IFNa activity in-vitro. BR J Haematol; 79(suppl 1):81-83. 1991.

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MISCELLANEOUS CANCER RESEARCH

Bone

Lea MA., Randolph VM. Induction of reported gene expression by inhibitors of histone deacetylase. Anticancer Res. 18(4A):2717-22. July-Aug 1998.

Lea M.A., and Randolph V.M. Histone hyperacetylation as a mechanism of action for phenylbutyrate on cancer cells. Proceedings of the AACR. Vol. 39, Mar 1998.

Shack S., Chen L., Miller A., Danesi R., Samid D. Increased Susceptibility of ras-tranformed Cells to Phenylacetate is Associated with Inhibition of p21ras Isoprenylation and Phenotypic Reversion. Int. J. Cancer., 63:124-129. 1995.

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Cervical

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

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Gastric

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

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Head & Neck

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

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Intestinal

Agarwal B., Halmos B., Prorvia P., Swaroop P. et al. Sodium Phenylacetate Inhibits Proliferation and Induces Apoptosis in Intestinal Epithelial Cell by Mechanisms Other than Inhibition of Isoprenylation. Proceedings of the AACR, Apr 1999.

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Kidney

Samid D., Wells M., Greene ME, Shen, W., Palmer CN, Thibault, A. Peroxisome Proliferator-activated Receptor Gamma as a Novel Target in Cancer Therapy: Binding and Activation by an Aromatic Fatty Acid with Clinical Antitumor Activity. Clin Cancer Res. 6(3):933-41. Mar 2000.

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Lung

Samid D, Hudgins WR, et al. Phenylacetate and derivatives: simple compounds with complex antitumor activities. Proceedings of the AACR, Apr 1994.

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

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Nervous System

Boudoulas S., Lush RM., McCall N.A., Samid D., Reed E., Figg WD. Plasma Protein Binding of Phenylacetate and Phenylbutyrate, Two Novel Antineoplastic Agents. Ther Drug Monit., 18:714-720. Dec 1996.

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Ovarian

Melichar B., Ferrandina G., Kudelka AP, et al. Growth Inhibitory Effects of Aromatic Fatty Acids on Ovarian Tumor Cell Lines. Clin Cancer Res., 4:3069-3076 Dec 1998.

Cheng Y., Lotan D., Lotan R. Expression of a Novel Retinoic Acid-inducible Gene (RAIG01) in Various Tumor Cell Lines and its Regulation by Retinoid, Butyrate, and Phenylacetate. Proceedings of the AACR, Mar 1998.

Ferrandina G., Melichar B., Loercher AE, et al. Growth Inhibitory Effects of Sodium Phenylacetate (NSC3039) on Ovarian Carcenoma Cell in Vitro. Cancer Res., 57(19): 4309-15. Mar 1997.

Shack S., Miller A., Liu L., Thibault A., Prasanna P., Samid D. Vulnerability of Multidrug-resistant Tumor Cells to the Aromatic Fatty Acids Phenylacetate and Phenylbutyrate. Clin. Cancer Res., 2:865-872. May 1996.

Shack S, Lui L, Miller A, Thibault A, Prasanna P, Samid D. Differentiation therapy using the aromatic fatty acids Phenylacetate and phenylbutyrate as an alterative treatment for multiple drug resistant tumors: in vitro studies. Proceedings of the AACR, Mar. 1995.

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Pancreatic

Harrison L., Wojciechowicz, D., Brennan, M., Paty, P. Phenylacetate Inhibits Isoprenoid Biosynthesis and Suppresses Growth of Human Pancreatic Carcinoma. Surgery, 124 (3):541-50. Sep 1998.

Wick M., Mangold G., Dexter D., Perkins W., Eckhardt G., Von Hoff D. Combination Drug Study with Sodium Phenylacetate and Gemcitabine Against the MiaPaCa Human Pancreatic Cancer Xenograft. Proceedings of the AACR, Mar 1997.

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Renal

Carducci M., Bowling MK., Eisenberger M., Sinibaldi V., Chen T.L., Noe D., Grochow L., Donehower R. Phenylbutyrate (PB) for Refractory Solid Tumors: Phase I Evaluation of Continuous Oral PB Exposure. Proceedings of the AACR, Mar 1998.

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Thyroid

Kebebew E., Wong M.G., Siperstein A.E., Duh, Q-Y and Clark, O.H. Phenylacetate Inhibits Growth and Vascular Endothelial Growth Factor Secretion in Human Thyroid Carcinoma Cells and Modulates Their Differentiated Function. J Clin Endocrin Metab. 84(8):2840-7. Aug 1999.

Carducci M., Bowling MK., Eisenberger M., Sinibaldi V., Chen T.L., Noe D., Grochow L., Donehower R. Phenylbutyrate (PB) for Refractory Solid Tumors: Phase I Evaluation of Continuous Oral PB Exposure. Proceedings of the AACR, Mar 1998.

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General

Gore SD., Carducci MA., Modifying histones to tame cancer: clinical development of sodium phenylbutyrate and other histone deacetylace inhibitors. Expert Opin Investig Drugs. (12):2923-34. Dec 2000.

Hommes, F.A. The Assay of Phenylacetic Acid and 4-phenylbutyric Acid in Physiological Fluids. Clin Chim Acta. 284(1):109-11. Jun 1999.

Darmanun D., Welch S., Rini A., et al Phenylbutyrate-induced Glutamine Depletion in Humans: Effect on Leucine Metabolism. Am J Physiol 274(5 Pt 1): E801-7. May 1998.

Samid D., Kulkarni M., Liu L., Thibault A. The Nuclear Receptors, PPARa and PPARg, are Molecular Targets of the Differentiation Inducers Phenylacetate and Phenylbutyrate. Proceedings of the AACR, Mar 1997.

Samid D, Hidgins WR, Shack S, Liu L, Prasanna P, Myers CE. Phenylacetate and phenylbutyrate as novel, nontoxic differentiation inducers. Adv. Exp. Med. Biol., 400A:501-505, 1997.

Thibault A, Figg WD, Samid D. A phase I study of the differentiating agent phenylbutyrate in patients with cancer. Proceedings for ASCO, 15:A1539, 1996.

Chen C, Shack S, Miller AC, Dannesi R, Samid D. Increased susceptibility of ras-transformed cells to Phenylacetate (PA) is associated with inhibition of p21ras isoprenylation and phenotypic reversion. Proceedings of the AACR, Mar 1995.

Johnson DE, Ochjeng J, Gibbs M, et al. Antiprolifeative activity of Phenylacetate acid dervicatives against MCF-7 cells. Proceedings of the AACR, Mar 1995.

Newmark H., Young C. Butyrate and Phenylacetate as Differentiating Agents: Practical Problems and Opportunities. J. Cell. Biochem,, Suppl. 22:247-253. 1995.

Piscitelli S., Thibault A., Figg W., et al. Disposition of Phenylbutyrate and its Metabolites, Phenylacetate and Phenylacetylglutamine. J. Clin. Pharmacol., 35:368-373. 1995.

Hudgins WR, Pineau T, Sher T, Gonzoles F, Myers CE, Samid D. Anticancer activity of Phenylacetate and related aromatic fatty acids: correlation with lipophilicity to activate a nuclear receptor. Proceedings of the AACR, Apr 1994.

Thibault A., Figg W., McCall N., Samid S., Myers C., Cooper M. A Simultaneous Assay of the Differentiation Agents Phenylacetic Acid and Phenylbutyric Acid, and one of their Metabolites, Phenylacetylglutamine, by Reversed-phase, High Performance Liquid Chromatography. J. Liq. Chrom., 17:2895-1900. 1994.

Samid D, Kulkami M, Lui L, Thibault A. The nuclear receptors, PPARa and PPARg, are molecular targets of the differentiation inducers Phenylacetate and phenylbutyrate. Proceedings of the AACR, 38:A3024, 1994.

Berg S., Serabe B., Aleksic A., Bomgaars L., McGuffey L., Dauser R., Durfee J., Nuchtern J., Blaney S. Pharmacokinetics and cerebrospinal fluid penetration of phenylacetate and phenylbutyrate in the nonhuman primate. Cancer Chemother Pharmacol. 47: 385-390. February 2001.

Gilbert J., Baker S., Zabelina Y., Grossman S., Carducci M. Anticonvulsant Therapy Increases the Clearance of Oral Phenylbutyrate. Proceedings of the AACR. Vol. 42, March 2001.

Gore SD., Baylin SB., Carducci MA., Cameron EE., Gilbert J., Miller CB., Herman JG. Sequential DNA Methyltransferase and Histone Deacetylase Inhibition to Re-express Silenced Genes: Pre-Clinical and Early Clinical Modeling. Proceedings of the AACR. Vol. 42, March 2001.


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SICKLE CELL ANEMIA, THALASSEMIA & COOLEY'S ANEMIA

Hoppe C., Vichinsky E., Lewis B., Foote D., Styles L. Hydroxyurea and Sodium Phenylbutyrate Therapy in Thalassemia Intermedia. Am J Hematol., 62(4):221-7. Dec 1999.

MacMillan ML, Fouladi M., Nisbet-Brown E., Waye JS, Oliveri, NF Treatment of Two Infants with Cooley's Anemia with Sodium Phenylbutyrate. Ann N Y Acad Sci., 850:452-4. Jun 1998.

Dover GJ Hemoglobin Switching Protocols in Thalassemia. Experience with Sodium Phenylbutyrate and Hydroxyurea. Ann N Y Acad Sci., 850:80-6. Jun 1998.

Oliveri NF, Rees, DC, Ginder GD, Thein SL, Waye JS. Elimination of Transfusions Through Induction of Fetal Hemoglobin Synthesis in Cooley's Anemia. Ann N Y Acad Sci., 850:100-9. Jun 1998.

Olivieri N.F., Rees D.C., Ginder G.D., et al. Treatment of Thalassemia Major with Phenylbutyrate and Hydroxyurea. Lancet., 350: 491-492. Aug 1997.

Hudgins W.R., Fibach E., Safaya S., Rieder R.F., Miller, A.C., Samid D. Transcriptional Upregulation of g-Globin by Phenylbutyrate and Analogous Aromatic Fatty Acids. Biochem Pharmacol., 52: 1227-1233. Oct 1996.

Collins A.F., Pearson H.A., Giardina P., McDonagh KT., Brusilow SW., Dover G.J. Oral Sodium Phenylbutyrate Therapy in Homozygous b Thalassemia: A Clinical Trial. Blood., 85: 43-49. 1995.

Dover G.J., Brusilow, S., Charache S. Induction of Fetal Hemoglobin Production in Subjects with Sickle Cell Anemia by Oral Sodium Phenylbutyrate. Blood 184(1):339-43. Jul 1994.

Fibach E., Prasanna P., Rodgers G., Samid D. Enhanced Fetal Hemoglobin Production by Phenylacetate and 4-Phenylbutyrate in Erythroid Precursors Derived from Normal Donors and Patients with Sickle Cell Anemia and b Thalassemia. Blood 82:2203-09. 1993.

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Excerpts From Books and Articles

Dover D., Brusilow S., Samid D. Increased Fetal Hemoglobin in Patients Receiving Sodium 4-Phenylbutyrate. New Eng. J. Med., 327:569-570, Oct 1992.

Leary, W.E. Cancer Drug Also Helps in Treating Sickle Cell Anemia, Researchers Say. Atlanta Journal Sect E10. Aug 1992.

Smigel K. Non-toxic Drug Being Tested to Treat Cancer and Anemias. J Natl Cancer Inst. 84(18):1398-9. Sep 1992.

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UREA CYCLE DISORDER/ ORNITHINE TRANSCARBAMYLASE DEFICIENCY

Batchaw ML., MacArthur RB, Tuchman M., Alternative pathway therapy for urea cycle disorders: twenty years later. J Pediatr., 138 (1 Suppl):S46-54; discussion S-54-5. Jan 2001.

Praphanphoj V, Boyadjiev SA, Waber LJ, Brusilow SW, Geraghty MT. Three caces of intravenous sodium benzoate and sodium Phenylacetate toxicity occurring in the treatment of acute hyperammonaemia. J. Inherit Metab. Dis., 23:129-136. 2000.

Brunquell P., Tezcan K., DiMarion FJ, Electroencephalographic findings in ornithine transcarbamylase deficiency. J Child Neurol., 14(8):533-6. Aug 1999.

Yudkoff M., Daikhin Y., Nissim I., Jaward A., Wilson J., Batshaw M., In vivo nitrogen metabolism in ornithine transcarbamylase deficiency. J Clin Invest., 98(9)2167-73. Nov 1996.

Maestri NE., Brusilow SW., Clissold DB., Bassett SS., Long-term treatment of girls with ornithine transcarbamylase deficiency. N Engl J Med., 19; 335(12):855-9. Sep 1996.

Brusilow SW, Maestri NE. Urea cycle disorders: Diagnosis, pathophysiology, and therapy. Adv. Pediatr., 43:127-170. 1996.

Maestri NE, Clissold DB, Brusilow SW. Long-term survival of patients with argininosuccinate synthetase deficiency. The Journal of Pediatrics, 929-935. Dec 1995.

bBrusilow SW., Finkelstien J., Restoration of nitrogen homeostacis in man with ornithine transcarbamylase deficiency. Metabolism., 42(10):1336-9. Oct 1993.

Brusilow S. Phenylacetylglutamine May Replace Urea as a Vehicle for Waste Nitrogen Excretion. Ped. Res., 29(2):147-150. 1991.

Maestri N., Hauser E., Bartholomew D., Brusilow S. Prospective Treatment of Urea Cycle Disorders. J Ped., 119(6):923-928. 1991.

Tuchman M., Knopman DS., Shih VE., Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Arch Neurol., 47(10):1134-7. Oct 1990.

Finkelstein J.E., Hauser E.R., Leonard C.O., Brusilow S.W. Late-onset Ornithine Transcarbamylase Deficiency in Male Patients. J Ped., 117(6):897-902. 1990.

Rowe P., Newman S., Brusilow S. Natural History of Symptomatic Partial Ornithine Transcarbamylase Deficiency. N Engl J Med., 314:541-547. 1986.

Brusilow S., Danney M., Waber L., Batshaw M., et al Treatment of Episodic Hyperammonemia in Children with Inborn Errors of Urea Synthesis. N Engl J Med., 310:1630-34. Jun 1984.

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Excerpt From Books and Articles

Redonnet-Vernhet I., Rouanet F., Pedespan JM, Hocke, C., Parrot F., A Successful Pregnancy in a Heterozygote for OTC Deficiency Treated with Sodium Phenylbutyrate. Neurology., 54(4):1008. Feb 2000.

Sodium Phenylbutyrate for Urea Cycle Enzyme Deficiencies. Med Lett Drugs Ther., 38(988):105-6. Nov 1996.

Gutteridge C, Kuhn RJ. Compatibility of 10% sodium Benzoate plus 10% sodium Phenylacetate with various flavored vehicles. Am J Hosp Pharm; 2508-2509. 1994.

Brusilow S. Treatment of Urea Cycle Disorders. Treatment of Genetic Disease (RJ Desknick ed) Churchill-Livingston, p79-94. 1991.

Rubenstein J.L.R., Johnston K., Elliot G.R., Brusilow S.R. Haloperidol-induced Hyperammonaemia in a Child with Citrullinaemia. J. Inher. Metab. Dis., 13: 754-755. 1990.

Batshaw M.L., Brusilow S.W. Evidence of Lack of Toxicity of Sodium Phenylacetate and Sodium Benzoate in Treating Urea Cycle Enzymopathies. J. Inher. Metab. Dis., 4:231. 1981.

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BENIGN PROSTATE HYPERPLASIA (BPH)

Lipschutz J.H., Samid D, Cunha G.R. Phenylacetate is an Inhibitor of Prostatic Growth and Development in Organ Culture. J Urol., 155:1762-1770. May 1996.

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CYSTIC FIBROSIS

McGrath-Morrow SA, Stahl JL. G(1) Phase growth arrest and induction of p21(Waf1/Cip1/Sdi1) in IB3-1 cells treated with 4-sodium phenylbutyrate. J Pharmacol Exp Ther., 294(3):941-7. Sep 2000.

Rubenstein R.C, Zeitlin P.L., Sodium 4-phenylbutyrate Downregulates Hsc70: Implications for Intracellular Trafficking of DF508-CFTR. Am J Physiol Cell Physiol., 278(2):C259-67. Feb 2000.

Zeitlin PL, Pharmacological Restoration of DeltaF508 CFTR-mediated Chloride Current. Kidney Int., 57(3):832-7. Mar 2000.

Loffing J., Moyer B.D., Reynolds D., Stanton B.A., PBA Increases CFTR Expression but at High Doses Inhibits C1(-) Secretion in Calu-3 Airway Epithelial Cells. Am J. Physiol., 277(4 Pt 1):L700-8. Oct 1999.

Rubenstein R.C., Zeitlin P.L., A Pilot Clinical Trial of Oral Sodium 4-phenylbutyrate (Buphenyl) in DF508-Homozygous Cystic Fibrosis Patients: Partial Restoration of Nasal Epithelial CFTR Function. Am J Respir Crit Care Med., 157(2):484-90. Feb 1998.

Rubenstein R.C., Zeitlin, P.L. Use of Protein Repair Therapy in the Treatment of Cystic Fibrosis. Curr Opin Pediatr., 10(3):250-5. Jun 1998.

Rubenstein R.C., Egan M.E., Zeitlin P.L. In vitro Pharmacologic Restoration of CFTR-mediated Chloride Transport with Sodium 4-Phenylbutyrate in Cystic Fibrosis Epithelial Cells Containing DF508- CFTR. J Clin Invest., 100:2457-2465. Nov 1997.

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Excerpts From Books and Articles

Bradbury N.A., Focus on "Sodium 4-phenylbutyrate Downregulates Hsc70: Implications for Intracellular Trafficking of DF508-CFTR." Am J Physiol Cell Physiol., 278(2):C257-8. Feb 2000.

Friend, T. Treatment of Cystic Fibrosis on the Fast Track. USA TODAY, 7D. Apr 1998.

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ADRENOLEUCODYSTROPHY (ALD)

Heming W, Kemp S, McGuinness MC, Moser AB, Smith KD. Pharmacological induction of Peroxisomes in Peroxisome Biogenesis Disorders. Ann Neurol, 47:286-296. 2000.

Van Geel BM, Assies J., Haverkort EB, Koelman JH, Progression of Abnormalities in Adrenomyeloneuropathy and Neurologically as Asymptomatic X-linked Adrenoleucodystrophy Despite Treatment with "Lorenzo's Oil" J Neurosurg Psych., 67(3):290-9. Sep 1999.

Kemp S., Wei H.M., Lu J.F., et al Gene Redundancy and Pharmacological Gene Therapy: Implications for X-link Adrenoleucodystrophy. Nat Med., 4(11):1261-8. Nov 1998.

Excerpts From Books and Articles

Wanders, R.J.A. A Happier Sequel to Lorenzo's Oil? Nat Med., 4(11):1245-6. Nov 1998.

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MISCELLANEOUS

McGrath-Morrow SA, Stahl JL. G(1) Phase growth arrest and induction of p21(Wafl/Cip/Sdi1) in IB3-1 cells treated with 4-sodium phenylbutyrate. J Pharmacol Exp. Ther., 294(3):941-7. Sep 2000.

Burrows J.A., Willis L.K., Perlmutter D.H. Chemical Chaperones Mediate Increased Secretion of Mutant a1-antitrypsin (a1-AT) Z: A Potential Pharmacological Strategy for Prevention of Liver Injury and Emphysema in a1-AT Deficiency. PNAS, 97(4):1796-1801. Feb 2000.

Hommes FA. The assay of phenylacetic acid and 4-phenylbutyric acid in physiological fluids. Clinica Chimica Acta, 284: 109-111. 1999.

Darmaun D., Welch S., Rini A., Sager B.K., Altomare A., Morey W.H. Phenylbutyrate-induced glutamine depletion in humans: effect on leucine metabolism. Am. J. Physiol.(Glutamine Depletion and Protein Catabolism) E801-E806, 28 Jan. 1998.

Boudoulas S., Lush R.M., McCall N.A., Samid D., Reed E., Figg W.D. Plasma Protein Binding of Phenylacetate and Phenylbutyrate, Two Novel Antineoplastic Agents. Therapeutic Drug Monitoring 18:714-720, 1996.

McCall N., Samid D., Figg W. Stability of Phenylacetate and Phenylbutyrate in Parenteral Large-volume Sterile Water. Drug Stability, 1:54-58. 1995.

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