Department of Pediatrics

Education

• PhD, Eotvos Lorand University, 1988
• MS, Eotvos Lorand University, 1985

Recent Publications view all (34)

Publication Co-Authors

David A. Rudnick, M.D., Ph.D.

Stephen C. Pak, Ph.D.

David Perlmutter, M.D.

Gary A. Silverman, M.D., Ph.D.

Washington University has a long tradition of collaboration amongst colleagues.The faculty above have co-authored publications with Tunda Hidvegi, Ph.D.

1. David A Rudnick, Jiansheng Huang, Tunda Hidvegi, Andrew S Chu, Pamela Hale, Admire Munanairi, Dennis J Dietzen, Paul F Cliften, Eric Tycksen, Andrew J Lutkewitte, Brian N Finck, Stephen C Pak, Gary A Silverman, David H Perlmutter. Regulation of PGC1α downstream of the insulin signaling pathway plays a role in the hepatic proteotoxicity of mutant α1-antitrypsin deficiency variant Z Gastroenterology. 2022. doi: Gastroenterology . 2022 Mar 14;S0016-5085(22)00261-X. doi: 10.1053/j.gastro.2022.03.010. In Press Journal Pre-proof.  PMCID:35301011  PMID:35301011 
2. Wang Y, Cobanoglu MC, Li J, Hidvegi T, Hale P, Ewing M, Chu AS, Gong Z, Muzumdar R, Pak SC, Silverman GA, Bahar I, Perlmutter DH. An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z PLoS One. 2019;14(1):e0209748. doi:10.1371/journal.pone.0209748. eCollection 2019  PMID: 30673724 
3. Mukherjee A, Hidvegi T, Araya P, Ewing M, Stolz DB, Perlmutter DH. NFκB mitigates the pathological effects of misfolded α1-antitrypsin by activating autophagy and an integrated program of proteostasis mechanisms. Cell Death Differ. 2018. PMID:29795336 
4. Hidvegi T, Stolz DB, Alcorn JF, Yousem SA, Wang J, Leme AS, Houghton AM, Hale P, Ewing M, Cai H, Garchar EA, Pastore N, Annunziata P, Kaminski N, Pilewski J, Shapiro SD, Pak SC, Silverman GA, Brunetti-Pierri N, Perlmutter DH. Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy. J Biol Chem. 2015;290(50):29742-57. doi:10.1074/jbc.M115.691253  PMCID:PMC4705969  PMID:26494620 
5. Li J, Pak SC, O'Reilly LP, Benson JA, Wang Y, Hidvegi T, Hale P, Dippold C, Ewing M, Silverman GA, Perlmutter DH. Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency. PLoS One. 2014;9(1):e87260. doi:10.1371/journal.pone.0087260  PMCID:PMC3909079  PMID:24498058 
6. Ding J, Yannam GR, Roy-Chowdhury N, Hidvegi T, Basma H, Rennard SI, Wong RJ, Avsar Y, Guha C, Perlmutter DH, Fox IJ, Roy-Chowdhury J. Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes. J Clin Invest. 2011;121(5):1930-4. doi:10.1172/JCI45260  PMCID:PMC3083768  PMID:21505264 
7. Hidvegi T, Mukherjee A, Ewing M, Kemp C, Perlmutter DH. The role of autophagy in alpha-1-antitrypsin deficiency. Methods Enzymol. 2011;499:33-54. doi:10.1016/B978-0-12-386471-0.00003-1  PMID:21683248 
8. Hidvegi T, Ewing M, Hale P, Dippold C, Beckett C, Kemp C, Maurice N, Mukherjee A, Goldbach C, Watkins S, Michalopoulos G, Perlmutter DH. An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis. Science. 2010;329(5988):229-32. doi:10.1126/science.1190354  PMID:20522742 
9. Hidvegi T, Mirnics K, Hale P, Ewing M, Beckett C, Perlmutter DH. Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency. J Biol Chem. 2007;282(38):27769-80. doi:10.1074/jbc.M704330200  PMID:17635928 
10. Kamimoto T, Shoji S, Hidvegi T, Mizushima N, Umebayashi K, Perlmutter DH, Yoshimori T. Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity. J Biol Chem. 2006;281(7):4467-76. doi:10.1074/jbc.M509409200  PMID:16365039 
11. Hidvegi T, Schmidt BZ, Hale P, Perlmutter DH. Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response. J Biol Chem. 2005;280(47):39002-15. doi:10.1074/jbc.M508652200  PMID:16183649 
12. Teckman JH, Burrows J, Hidvegi T, Schmidt B, Hale PD, Perlmutter DH. The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes. J Biol Chem. 2001;276(48):44865-72. doi:10.1074/jbc.M103703200  PMID:11577074 
13. Circolo A, Garnier G, Fukuda W, Wang X, Hidvegi T, Szalai AJ, Briles DE, Volanakis JE, Wetsel RA, Colten HR. Genetic disruption of the murine complement C3 promoter region generates deficient mice with extrahepatic expression of C3 mRNA. Immunopharmacology. 1999;42(1-3):135-49. PMID:10408374 
14. Schmidt BZ, Fowler NL, Hidvegi T, Perlmutter DH, Colten HR. Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency. J Biol Chem. 1999;274(17):11782-8. PMID:10206995 
15. Dervaderics M, Hidvégi T, Schmidt B, Füst G, Varga L. Ragweed allergy: correlation between skin reactivity and in vitro complement activation. Immunol Lett. 1998;64(2-3):119-23. PMID:9870662 
16. Gönczi Z, Varga L, Hidvégi T, Schmidt B, Pánya A, Kókai M, Füst G. The severity of clinical symptoms in ragweed-allergic patients is related to the extent of ragweed-induced complement activation in their sera. Allergy. 1997;52(11):1110-4. PMID:9404564 
17. Matsumoto M, Fukuda W, Circolo A, Goellner J, Strauss-Schoenberger J, Wang X, Fujita S, Hidvegi T, Chaplin DD, Colten HR. Abrogation of the alternative complement pathway by targeted deletion of murine factor B. Proc Natl Acad Sci U S A. 1997;94(16):8720-5. PMCID:PMC23097  PMID:9238044 
18. Prohászka Z, Nemes J, Hidvégi T, Tóth FD, Kerekes K, Erdei A, Szabó J, Ujhelyi E, Thielens N, Dierich MP, Späth P, Ghebrehiwet B, Hampl H, Kiss J, Arlaud G, Füst G. Two parallel routes of the complement-mediated antibody-dependent enhancement of HIV-1 infection. AIDS. 1997;11(8):949-58. PMID:9223728 
19. Hidvégi T, Berrens L, Varga L, Marañon F, Schmidt B, Kirschfink M, Füst G. Comparative study of the complement-activating and specific IgE-binding properties of ragweed pollen allergen. Clin Exp Immunol. 1997;108(1):122-7. PMCID:PMC1904628  PMID:9097920 
20. Hidvégi T, Schmidt B, Varga L, Dervaderics M, Lantos A, Gönczi Z, Barok J, Otos M, Kirschfink M, Späth P. In vitro complement activation by ragweed allergen extract in the sera of ragweed allergic and non-allergic persons. Immunol Lett. 1995;48(1):65-71. PMID:8847094 
21. Prohászka Z, Hidvégi T, Ujhelyi E, Stoiber H, Dierich MP, Süsal C, Füst G. Interaction of complement and specific antibodies with the external glycoprotein 120 of HIV-1. Immunology. 1995;85(2):184-9. PMCID:PMC1383879  PMID:7642209 
22. Füst G, Dierich MP, Hidvégi T. Role of humoral factors in the progression of HIV disease. Immunol Today. 1995;16(4):167-9. PMID:7734041 
23. Füst G, Prohászka Z, Hidvégi T, Thielens N, Arlaud G, Tóth FD, Kiss J, Dierich MP, Ujhelyi E. Competition of complement proteins and specific antibodies for binding to HIV-1 envelope antigens. Acta Microbiol Immunol Hung. 1994;41 Suppl:27-31. PMID:8075944 
24. Rácz Z, Baróti C, Hidvégi T, Skopál J, Thék M. Quality of plasma separated from (buffy coat + plasma). An alternative way of blood processing. Acta Haematol Pol. 1994;25(1):47-53. PMID:8209614 
25. Hidvégi T, Prohászka Z, Ujhelyi E, Thielens NM, Dierich MP, Hampl H, Arlaud G, Nagy K, Füst G. Studies on the mechanism of complement-mediated inhibition of antibody binding to HIV gp41. Clin Exp Immunol. 1993;94(3):490-3. PMCID:PMC1534428  PMID:8252810 
26. el Jarrah F, Hidvégi T, Ujhelyi E, Gyapay G, Salavecz V, Origer JJ, Hollán S, Füst G. Complement activation decreases the ability of HIV transmembrane envelope protein to bind to specific antibody. AIDS. 1992;6(9):1050-1. PMID:1388901 
27. Füst G, Ujhelyi E, Hidvégi T, Pálóczi K, Mihalik R, Hollán S, Nagy K, Kirschfink M. The complement system in HIV disease. Immunol Invest. 1991;20(2):231-41. PMID:1864641 
28. Gregorek H, Kokai M, Hidvégi T, Füst G, Sabbouh K, Madaliński K. Concentration of C1 inhibitor in sera of healthy blood donors as studied by immunoenzymatic assay. Complement Inflamm. 1991;8(5-6):310-2. PMID:1802549 
29. Hidvégi T, Varga L, Falus A, Kirschfink M, Kókai M, Tuan NA, Füst G. Differences in the complement activation induced by preformed and nascent immune complexes. Complement Inflamm. 1991;8(1):43-9. PMID:2049935 
30. Falus A, Fehér KG, Walcz E, Brozik M, Füst G, Hidvégi T, Fehér T, Merétey K. Hormonal regulation of complement biosynthesis in human cell lines--I. Androgens and gamma-interferon stimulate the biosynthesis and gene expression of C1 inhibitor in human cell lines U937 and HepG2. Mol Immunol. 1990;27(2):191-5. PMID:2157144 
31. Falus A, Rokita H, Walcz E, Brozik M, Hidvégi T, Merétey K. Hormonal regulation of complement biosynthesis in human cell lines--II. Upregulation of the biosynthesis of complement components C3, factor B and C1 inhibitor by interleukin-6 and interleukin-1 in human hepatoma cell line. Mol Immunol. 1990;27(2):197-201. PMID:2157145 
32. Hidvégi T, Ermolin GA, Efremov EE, Dikov MM, Kurmanova LV, Vnashenkova GV, Merkulova MV, Kókai M, Panya A, Füst G. FN-C1q and C1 INH C1r-C1s complexes as indicators of complement activation in patients with chronic lymphocytic leukaemia. Immunol Lett. 1989;22(1):1-6. PMID:2550362 
33. Hidvégi T, Füst G, Rajnavölgyi E, Kulics J, Gergely J. Non-covalently bound C3 enhances lysis of rabbit erythrocytes through the alternative pathway. Immunology. 1985;56(4):735-41. PMCID:PMC1453793  PMID:3935572 
34. Hidvégi T, Fehér GK, Fehér T, Koó E, Füst G. Inhibition of the complement activation by an adrenal androgen, dehydroepiandrosterone. Complement. 1984;1(4):201-6. PMID:6242390