Pubmed search for Dr. Judith Fridovich-Keil


1. Serhan, C.N., J. Fridovich, E. Goetzl, P. Dunham, and G. Weissmann. (1982).

Leukotriene B4 and Phosphatidic Acid are Calcium Ionophores. (Studies Employing

Arsenazo III in Liposomes). J. Biol. Chem. 257:4746-4752.


2. Bond, J.F., J.L. Fridovich-Keil, L. Pillus, R. Mulligan, and F. Solomon. (1986). A Chicken-

Yeast Chimeric Beta-Tubulin Protein is Incorporated into Mouse Microtubules In Vivo.

Cell 44:461-468.


3. Fridovich-Keil, J.L., J.F. Bond, and F. Solomon. (1987). Domains of Beta-Tubulin

Essential for Conserved Functions In Vivo. Mol. Cell. Biol. 7:3792-3798.


4. Dezube, B.J., J.L. Fridovich-Keil, I. Bouvard, R. Lange, and A.B. Pardee. (1990).

Pentoxifylline and wellbeing in patients with cancer. The Lancet 335:662 (letter).


5. Bradley, D.W., Q.-P. Dou, J.L. Fridovich-Keil, and A.B. Pardee. (1990). Transformed and

Non-Transformed Cells Differ in Stability and Cell Cycle Regulation of a Binding Activity

to the Thymidine Kinase Promoter. Proc. Natl. Acad. Sci. USA. 87:9310-9314.


6. Fridovich-Keil, J.L., J. Gudas, Q.-P. Dou, I. Bouvard, and A.B. Pardee. (1991). Growth-

Responsive Expression from the Murine Thymidine Kinase Promoter: Genetic Analysis

of DNA Sequences. Cell Growth and Differentiation. 2:67-76.


7. Dou, Q.-P., J.L. Fridovich-Keil, and A.B. Pardee. (1991). Inducible Proteins Binding to

the Murine Thymidine Kinase Promoter in Late G1/ S Phase. Proc. Natl. Acad. Sci. USA.



8. Fridovich-Keil, J.L., J.M. Gudas, I.B. Bryan, and A.B. Pardee. (1991). Improved

Expression Vectors for Eukaryotic Promoter/Enhancer Studies. .BioTechn. 11:572-9.


9. Gudas, J.M., J.L. Fridovich-Keil, M.W. Datta, J. Bryan, and A.B. Pardee. (1992)

Molecular characterization of the murine thymidine kinase-encoding gene and analysis

of transcription start point heterogeneity. Gene 118:205-216.


10. Leslie, N.D., E.B. Immerman, J.E. Flach, M. Florez, J.L. Fridovich-Keil, and L.J. Elsas.

(1992) The Human Galactose-1-P Uridyltransferase Gene. Genomics 14:474-80.


11. Dezube, B.J., M.L. Sherman, J.L. Fridovich-Keil, J. Allen-Ryan, and A.B. Pardee. (1993).

Down-regulation of tumor necrosis factor expression by pentoxifylline in cancer patients:

a pilot study. Cancer Immunol. Immunother. 36:57-60.


12. Fridovich-Keil, J.L. and S. Jinks-Robertson. (1993). A yeast expression system for

human galactose-1-phosphate uridyltransferase. Proc. Natl. Acad. Sci. USA. 90:398-



13. Gudas, J.M., J.L. Fridovich-Keil, and A.B. Pardee. (1993). Posttranscriptional Control of

Thymidine Kinase mRNA during the Cell Cycle. Cell Growth and Diff. 4:421-30.


14. Fridovich-Keil, J.L., P.J. Markell, J.M. Gudas, and A.B. Pardee. (1993). DNA sequences

required for serum-responsive regulation of the mouse thymidine-kinase promoter. Cell

Growth and Differentiation 4:679-687.


15. Elsas, L.J., P.P. Dembure, S. Langley, E.M. Paulk, L.N. Hjelm, and J.L. Fridovich-

Keil.(1994). A Common Mutation Associated with the Duarte Galactosemia Allele. Am.

J. Hum. Genet. 54:1030-1036.


16. Bradley, D.W., J.L. Fridovich-Keil, J.M. Gudas, and A.B. Pardee. (1994). Serumresponsive

expression from the murine thymidine kinase promoter is specifically

disrupted in a transformed cell line. Cell Growth and Differentiation 5:1137-1143.


17. Fridovich-Keil, J.L., S.D.Langley, L.A. Mazur, J.C. Lennon, P.P Dembure, and L.J. Elsas.

(1995) Identification and functional analysis of three distinct mutations in the human gal-

1-P uridyltransferase gene associated with galactosemia in a single family. Am. J. Hum.

Gen. 56:640-646.


18. Elsas, L.J., S. Langley, E. Steele, J. Evinger, J.L.Fridovich-Keil, A. Brown, R. Singh, P.

Fernhoff, L.N. Hjelm, and P.P. Dembure.(1995) Galactosemia: A Strategy to Identify

New Biochemical Phenotypes and Molecular Genotypes. Am. J. Hum. Gen. 56:630-639.


19. Fridovich-Keil, J.L., B.B. Quimby, L. Wells, L.A. Mazur, and J.P. Elsevier (1995).

Characterization of the N314D Allele of Human Galactose-1-Phosphate

Uridyltransferase Using a Yeast Expression System. Biochemical and Molecular

Medicine 56:121-130.


20. Elsevier, J.P., L. Wells, B.B. Quimby, and J.L Fridovich-Keil. (1996) Heterodimer

formation and activity in the human enzyme galactose-1-phosphate uridylyltransferase.

Proc. Natl. Acad. Sci. USA. 93:7166-7171.


21. Quimby, B.B., L. Wells, K.D. Wilkinson, and J.L. Fridovich-Keil. (1996) Functional

requirements of the active site position 185 in the human enzyme galactose-1-

phosphate uridylyltransferase. J. Biol. Chem. 271:26835-26842.


22. Elsevier, J.P. and J.L. Fridovich-Keil. (1996) The Q188R mutation in human galactose-1-

phosphate uridylyltransferase acts as a partial dominant negative. J. Biol. Chem.



23. Wells, L., and J.L. Fridovich-Keil. (1997) Biochemical characterization of the S135L

allele of galactose-1-phosphate uridylyltransferase associated with galactosemia. J.

Inher. Metab. Dis. 20:633-642.


24. Quimby, B.B., A. Alano, S. Almashanu, A.M.. DeSandro, T.M. Cowan, and J.L Fridovich-

Keil. (1997) Characterization of two mutations associated with epimerase-deficiency

galactosemia using a yeast expression system for human UDP-galactose-4-epimerase.

Am. J. Hum. Gen. 61:590-598.


25. Wohlers, T.M., N.C. Christacos, M. T. Harreman, and J.L. Fridovich-Keil. (1999)

Identification and characterization of a mutation in the human UDP-galactose-4-

epimerase (GALE) gene associated with generalized epimerase-deficiency

galactosemia. Am. J. Hum. Gen. 64:462-470.


26. Lang, B.D and J.L. Fridovich-Keil. (2000) Scp160p, a multiple KH-domain protein, is a

component of mRNP complexes in yeast. Nucleic Acids Res. 28:1576-1584.


27. Thoden, J.B., T.M. Wohlers, J.L. Fridovich-Keil, and H.M. Holden. (2000)

Crystallographic evidence for tyr157 functioning as the active site base in human UDPgalactose

4-epimerase. Biochemistry 39(19):5691-5701.


28. Crews, C., K.D. Wilkinson, L. Wells, C. Perkins, and J.L. Fridovich-Keil. (2000)

Functional consequence of substitutions at position 171 in human galactose-1-P

uridylyltransferase. J. Biol. Chem. 275:22847-53.


29. Christacos, N.C., M.J. Marson, L. Wells, K. Riehman, and J.L. Fridovich-Keil. (2000)

Subcellular Localization of galactose-1-phosphate uridylyltransferase in the yeast

Saccharomyces cerevisiae. Mol. Gen. and Metabolism 70:272-280.


30. Henderson, J.M., L. Wells, and J.L. Fridovich-Keil. (2000) Covalent heterogeneity of the

human enzyme galactose-1-phosphate uridylyltransferase. J Biol Chem. 275:30088-91.


31. Wohlers, T.M. and J.L. Fridovich-Keil. (2000) Studies of the V94M-substituted human

UDP-galactose-4-epimerase enzyme associated with generalized epimerase-deficiency

galactosemia. J. Inher. Metab. Dis 23:713-729.


32. Riehman, K., C. Crews, and J.L. Fridovich-Keil. (2001) Relationship between genotype,

activity, and galactose sensitivity in yeast expressing patient alleles of human galactose-

1-phosphate uridylyltransferase. J. Biol. Chem. 276(14):10634-40.


33. Thoden, J.B., T.M. Wohlers, J.L. Fridovich-Keil, and H.M. Holden (2001) Human UDPGalactose

4-epimerase: Accommodation of UDP-N-Acetylglucosamine Within the Active

Site. J. Biol. Chem. 276(18):15131-15136.


34. Thoden, J.B., T.M. Wohlers, J.L. Fridovich-Keil, and H.M. Holden (2001) Molecular basis

for severe epimerase-deficiency galactosemia: X-ray structure of the human V94Msubstituted

UDP-galactose-4-epimerase. J. Biol. Chem. 276(23):20617-20623.


35. Lang, B.D., A-M. Li, H.D. Black-Brewster, and J.L. Fridovich-Keil. (2001) The brefeldin A

resistance protein Bfr1p is a component of polyribosome-associated mRNP complexes

in yeast. Nucleic Acids Research 29(12):2567-2574.


36. Henderson, J.M., S.M. Huguenin, T.M. Cowan, and J.L. Fridovich-Keil. (2001) A PCRbased

method for detecting known mutations in the human UDP galactose-4'-epimerase

gene associated with epimerase-deficiency galactosemia. Clinical Genetics. 60:350-355.


37. Thoden, J.B., J.M. Henderson, J.L. Fridovich-Keil, and H.M. Holden. (2002) Structural

analysis of the Y299C Mutant of Escherichia coli UDP-galactose 4'-epimerase: teaching

an old dog new tricks. J Biol Chem.277(30):27528-34.


38. Christacos, N.C. and J.L. Fridovich-Keil. (2002) Impact of patient mutations on

heterodimer formation and function in human galactose-1-P uridylyltransferase.

Molecular Genetics and Metabolism 76(4):319.


39. Li, A-M., A. Watson, and J.L. Fridovich-Keil. (2003) Scp160p associates with specific

mRNAs in yeast. Nucleic Acids Research. 31(7):1830-1837.


40. Mendelsohn, B.A., C.A. Vargas, A-M. Li, A. Watson, K. Riehman, and J.L. Fridovich-Keil

(2003). Genetic and biochemical interactions between SCP160 and EAP1 in yeast.

Nucleic Acids Research. 31(20):5838-47.


41. Henderson, J.M., A. Watson, R. Sanders, J.B. Thoden, H.M. Holden, and J.L. Fridovich-

Keil (2004) Determinants of Function and Substrate Specificity in Human UDPGalactose

4’-Epimerase. J Biol Chem 279(31):32796-803.


42. Li, A-M, CA Vargas, M Brykailo, K Openo, A Corbett, and JL Fridovich-Keil (2004). Both

KH and non-KH domain sequences are required for polyribosome association of

Scp160p in yeast. Nucleic Acids Res. 32(16):4768-75.


43. Ross, K.L., C. Davis, and J.L. Fridovich-Keil (2004). Differential roles of the Leloir

pathway enzymes and metabolites in defining galactose sensitivity in yeast. Molecular

Genetics and Metabolism. 83(1-2):103-116.


44. Wasilenko, J., M.E. Lucas, J.B. Thoden, H.M. Holden, and J.L. Fridovich-Keil (2005).

Functional characterization of the K257R and G319E hGALE alleles found in patients

with ostensibly peripheral epimerase deficiency galactosemia. Molecular Genetics and

Metabolism 84(1):32-8.


45. Schulz, JM, KL Ross, K Malmstrom, M. Krieger, and JL Fridovich-Keil (2005). Mediators

of galactose sensitivity in UDP-galactose 4'-epimerase impaired mammalian cells. J.

Biol. Chem. 280(14):13493-502.


46. Openo, KK, JM Schulz, CA Vargas, CS Orton, MP Epstein, RE Schnur, F Scaglia, GT

Berry, GS Gottesman, C Ficicioglu, AE Slonim, RJ Schroer, C Yu, V Rangel, J Keenan,

K Lamance, and JL Fridovich-Keil (2006).Epimerase-deficiency galactosemia is not a

binary condition. Am J Hum Gen. 78(1):89-102.


47. Wasilenko, J. and J.L. Fridovich-Keil (2006). Relationship between UDP galactose 4'-

epimerase activity and galactose sensitivity in yeast. J. Biol. Chem. 281(13):8443-9.


48. Brykailo, M, AH Corbett, and JL Fridovich-Keil. (2007) Functional overlap between

conserved and diverged KH domains in Saccharomyces cerevisiae SCP160. Nucleic

Acids Res. 35(4):1108-18.


49. Brykailo, M, L McLane, JL Fridovich-Keil, and A Corbett. (2007) Analysis of a predicted

nuclear localization signal: implications for the intracellular localization and function of

the Saccharomyces cerevisiae RNA-binding protein Scp160. Nucleic Acids



50. Mumma, JO, JS Chhay, KL Ross, JS Eaton, KA Newell-Litwa, and JL Fridovich-Keil.

(2008) Distinct roles of gal-1P in galactose-mediated growth arrest of GALT-null and

GALE-null yeast. Molecular Genetics and Metabolism, 93:160-171.


51. Chhay, JS, KK Openo, JS Eaton, M Gentile, and JL Fridovich-Keil. (2008) A yeast model

reveals biochemical severity among members of a family segregating three variant

alleles of galactose-1-P uridylyltransferase. J. Inher Metab Dis. 31(1):97-107.


52. Chhay, JS, CA Vargas, TJ McCorvie, JL Fridovich-Keil, and DJ Timson. (2008) Analysis

of UDP-galactose 4'-epimerase mutations associated with the intermediate form of type

III galactosaemia. J Inher Metab Dis, 31(1):108-16.


53. Sanders, RD, JB Spencer, MP Epstein, SV Pollak, PA Vardhana, JW Lustbader, and JL

Fridovich-Keil. (2009) Biomarkers of ovarian function in girls and women with classic

galactosemia. Fertility and Sterility. 18(9):1624-32 (2008 Aug 4, Epub). * see follow-up

letter to the Editor in 2009 Aug;92(2):e30.


54. Carney, AE, RD Sanders, KR Garza, LA McGaha, JLH Bean, BW Coffee, JW Thomas,

DJ Cutler, N Kurtkaya, and JL Fridovich-Keil. (2009) Origins, distribution, and expression

of the Duarte-2 (D2) allele of galactose-1-P uridylyltransferase (GALT). Hum. Mol. Gen.



55. Kushner, RF, EL Ryan, JMI Sefton, RD Sanders, P Jumbo Lucioni, KH Moberg, and JL

Fridovich-Keil. A Drosophila melanogaster model of classic galactosemia. In revision.


56. Sanders, RD, JMI Sefton, KH Moberg, and JL Fridovich-Keil. UDP-galactose 4’

epimerase (GALE) is essential for development of Drosophila melanogaster. In revision.