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Article
2020

Mechanisms of action of autophagy modulators dissected by quantitative systems pharmacology analysis

Shi, Q., Pei, F., Silverman, G. A., Pak, S. C., Perlmutter, D. H., Liu, B. & Bahar, I., Apr 1 2020, In : International journal of molecular sciences. 21, 8, 2855.

Research output: Contribution to journalArticle

Open Access
2019

An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z

Wang, Y., Cobanoglu, M. C., Li, J., Hidvegi, T., Hale, P., Ewing, M., Chu, A. S., Gong, Z., Muzumdar, R., Pak, S. C., Silverman, G. A., Bahar, I. & Perlmutter, D. H., Jan 2019, In : PloS one. 14, 1, e0209748.

Research output: Contribution to journalArticle

Open Access
3 Scopus citations

NFκB mitigates the pathological effects of misfolded α1-antitrypsin by activating autophagy and an integrated program of proteostasis mechanisms

Mukherjee, A., Hidvegi, T., Araya, P., Ewing, M., Stolz, D. B. & Perlmutter, D. H., Mar 1 2019, In : Cell Death and Differentiation. 26, 3, p. 455-469 15 p.

Research output: Contribution to journalArticle

3 Scopus citations
2018

Business of dialysis and the role of the medical director

Scheel, P. J., Mar 1 2018, In : Seminars in Dialysis. 31, 2, p. 177-182 6 p.

Research output: Contribution to journalArticle

1 Scopus citations
2017

Quantitative assessment of cell fate decision between autophagy and apoptosis

Liu, B., Oltvai, Z. N., Baylr, H., Silverman, G. A., Pak, S. C., Perlmutter, D. H. & Bahar, I., Dec 1 2017, In : Scientific reports. 7, 1, 17605.

Research output: Contribution to journalArticle

Open Access
13 Scopus citations
2015

Deficient and null variants of SERPINA1 are proteotoxic in a Caenorhabditis elegans model of α1-antitrypsin deficiency

Cummings, E. E., O'Reilly, L. P., King, D. E., Silverman, R. M., Miedel, M. T., Luke, C. J., Perlmutter, D. H., Silverman, G. A. & Pak, S. C., Oct 29 2015, In : PloS one. 10, 10, e0141542.

Research output: Contribution to journalArticle

Open Access
5 Scopus citations

Does surgeon experience affect outcomes in pathologic stage i lung cancer?

Scheel, P. J., Crabtree, T. D., Bell, J. M., Frederiksen, C., Broderick, S. R., Krupnick, A. S., Kreisel, D., Patterson, G. A., Meyers, B. F. & Puri, V., Apr 1 2015, In : Journal of Thoracic and Cardiovascular Surgery. 149, 4, p. 998-1004.e1

Research output: Contribution to journalArticle

Open Access
11 Scopus citations

Enhancing autophagy with drugs or lung-directed gene therapy reverses the pathological effects of respiratory epithelial cell proteinopathy

Hidvegi, T., Stolz, D. B., Alcorn, J. F., Yousem, S. A., Wang, J., Leme, A. S., Houghton, A. M. G., Hale, P., Ewing, M., Cai, H., Garchar, E. A., Pastore, N., Annunziata, P., Kaminski, N., Pilewski, J., Shapiro, S. D., Pak, S. C., Silverman, G. A., Brunetti-Pierri, N. & Perlmutter, D. H., Dec 11 2015, In : Journal of Biological Chemistry. 290, 50, p. 29742-29757 16 p.

Research output: Contribution to journalArticle

Open Access
19 Scopus citations

IndUced Pluripotent Stem Cells Model Personalized Variations in Liver Disease Resulting From α1-Antitrypsin Deficiency

Tafaleng, E. N., Chakraborty, S., Han, B., Hale, P., Wu, W., Soto-Gutierrez, A., Feghali-Bostwick, C. A., Wilson, A. A., Kotton, D. N., Nagaya, M., Strom, S. C., Roy-Chowdhury, J., Stolz, D. B., Perlmutter, D. H. & Fox, I. J., Jul 1 2015, In : Hepatology. 62, 1, p. 147-157 11 p.

Research output: Contribution to journalArticle

46 Scopus citations
2014

A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency

O'Reilly, L. P., Long, O. S., Cobanoglu, M. C., Benson, J. A., Luke, C. J., Miedel, M. T., Hale, P., Perlmutter, D. H., Bahar, I., Silverman, G. A. & Pak, S. C., Oct 1 2014, In : Human molecular genetics. 23, 19, p. 5123-5132 10 p.

Research output: Contribution to journalArticle

Open Access
27 Scopus citations

An instrumental variable approach finds no associated harm or benefit with early dialysis initiation in the United States

Scialla, J. J., Liu, J., Crews, D. C., Guo, H., Bandeen-Roche, K., Ephraim, P. L., Tangri, N., Sozio, S. M., Shafi, T., Miskulin, D. C., Michels, W. M., Jaar, B. G., Wu, A. W., Powe, N. R., Boulware, E. L., Cook, C., Coresh, J., Kim, J., Liu, Y., Luly, J. & 18 others, McDermott, A., Scheel, P., Zhou, J., Collins, A., Foley, R., Gilbertson, D., Heubner, B., Herzog, C., St Peter, W., Nally, J., Arrigain, S., Jolly, S., Konig, V., Liu, X., Navaneethan, S., Schold, J., Zager, P. & Meyer, K., Jan 1 2014, In : Kidney International. 86, 4, p. 798-809 12 p.

Research output: Contribution to journalArticle

Open Access
20 Scopus citations
2011

Association between pregnancy complications and small-for-gestational-age birth weight defined by customized fetal growth standard versus a population-based standard

Odibo, A. O., Francis, A., Cahill, A. G., MacOnes, G. A., Crane, J. P. & Gardosi, J., Mar 1 2011, In : Journal of Maternal-Fetal and Neonatal Medicine. 24, 3, p. 411-417 7 p.

Research output: Contribution to journalArticle

46 Scopus citations
Open Access
24 Scopus citations
2010

Effect of placenta previa on fetal growth

Harper, L. M., Odibo, A. O., MacOnes, G. A., Crane, J. P. & Cahill, A. G., Oct 2010, In : American journal of obstetrics and gynecology. 203, 4, p. 330.e1-330.e5

Research output: Contribution to journalArticle

35 Scopus citations

Is chorionic villus sampling associated with hypertensive disorders of pregnancy?

Odibo, A. O., Singla, A., Gray, D. L., Dicke, J. M., Oberle, B. & Crane, J., Jan 1 2010, In : Prenatal Diagnosis. 30, 1, p. 9-13 5 p.

Research output: Contribution to journalArticle

16 Scopus citations

Leiomyomas at routine second-trimester ultrasound examination and adverse obstetric outcomes

Stout, M. J., Odibo, A. O., Graseck, A. S., MacOnes, G. A., Crane, J. P. & Cahill, A. G., Nov 2010, In : Obstetrics and gynecology. 116, 5, p. 1056-1063 8 p.

Research output: Contribution to journalArticle

85 Scopus citations

Single umbilical artery and its associated findings

Hua, M., Odibo, A. O., MacOnes, G. A., Roehl, K. A., Crane, J. P. & Cahill, A. G., May 1 2010, In : Obstetrics and gynecology. 115, 5, p. 930-934 5 p.

Research output: Contribution to journalArticle

67 Scopus citations

Ultrasound-detected subchorionic hemorrhage and the obstetric implications

Norman, S. M., Odibo, A. O., Macones, G. A., Dicke, J. M., Crane, J. P. & Cahill, A. G., Aug 2010, In : Obstetrics and gynecology. 116, 2, p. 311-315 5 p.

Research output: Contribution to journalArticle

25 Scopus citations
2009

Pregnancy loss rate after mid-trimester amniocentesis in twin pregnancies

Cahill, A. G., Macones, G. A., Stamilio, D. M., Dicke, J. M., Crane, J. P. & Odibo, A. O., Mar 2009, In : American journal of obstetrics and gynecology. 200, 3, p. 257.e1-257.e6

Research output: Contribution to journalArticle

45 Scopus citations
2008

Evaluating the rate and risk factors for fetal loss after chorionic villus sampling

Odibo, A. O., Dicke, J. M., Gray, D. L., Oberle, B., Stamilio, D. M., MacOnes, G. A. & Crane, J. P., Oct 1 2008, In : Obstetrics and gynecology. 112, 4, p. 813-819 7 p.

Research output: Contribution to journalArticle

Open Access
52 Scopus citations

Revisiting the fetal loss rate after second-trimester genetic amniocentesis: A single center's 16-year experience

Odibo, A. O., Gray, D. L., Dicke, J. M., Stamilio, D. M., Macones, G. A. & Crane, J. P., Mar 1 2008, In : Obstetrics and gynecology. 111, 3, p. 589-595 7 p.

Research output: Contribution to journalArticle

Open Access
125 Scopus citations
2003

An outbreak of Pseudomonas aeruginosa infections associated with flexible bronchoscopes

Srinivasan, A., Wolfenden, L. L., Song, X., Mackie, K., Hartsell, T. L., Jones, H. D., Diette, G. B., Orens, J. B., Yung, R. C., Ross, T. L., Merz, W., Scheel, P. J., Haponik, E. F. & Perl, T. M., Jan 16 2003, In : New England Journal of Medicine. 348, 3, p. 221-227 7 p.

Research output: Contribution to journalArticle

151 Scopus citations

Spontaneous renal artery dissection: Treatment with coil embolization

Mudrick, D., Arepally, A., Geschwind, J. F., Ronsivalle, J. A., Lund, G. B. & Scheel, P., Apr 1 2003, In : Journal of Vascular and Interventional Radiology. 14, 4, p. 497-500 4 p.

Research output: Contribution to journalArticle

25 Scopus citations
2002
29 Scopus citations

Extrahepatic biliary atresia: A disease or a phenotype?

Perlmutter, D. H. & Shepherd, R. W., Jun 2002, In : Hepatology. 35, 6, p. 1297-1304 8 p.

Research output: Contribution to journalArticle

107 Scopus citations

Fasting in α1-antitrypsin deficient liver: Consultative activation of autophagy

Teckman, J. H., An, J. K., Loethen, S. & Perlmutter, D. H., Nov 2002, In : American Journal of Physiology - Gastrointestinal and Liver Physiology. 283, 5 46-5, p. G1156-G1165

Research output: Contribution to journalArticle

64 Scopus citations
2001

A Naturally Occurring Nonpolymerogenic Mutant of α1-Antitrypsin Characterized by Prolonged Retention in the Endoplasmic Reticulum

Lin, L., Schmidt, B., Teckman, J. & Perlmutter, D. H., Sep 7 2001, In : Journal of Biological Chemistry. 276, 36, p. 33893-33898 6 p.

Research output: Contribution to journalArticle

Open Access
48 Scopus citations

Outcome of early hepatic portoenterostomy for biliary atresia

Volpert, D., White, F., Finegold, M. J., Molleston, J., DeBaun, M. & Perlmutter, D. H., May 7 2001, In : Journal of pediatric gastroenterology and nutrition. 32, 3, p. 265-269 5 p.

Research output: Contribution to journalArticle

36 Scopus citations
Open Access
120 Scopus citations
2000

Circulating serpin tumor markers SCCA1 and SCCA2 are not actively secreted but reside in the cytosol of squamous carcinoma cells

Uemura, Y., Pak, S. C., Luke, C., Çataltepe, S., Tsu, C., Schick, C., Kamachi, Y., Pomeroy, S. L., Perlmutter, D. H. & Silverman, G. A., Jul 20 2000, In : International Journal of Cancer. 89, 4, p. 368-377 10 p.

Research output: Contribution to journalArticle

60 Scopus citations

Cohort study of the treatment of severe HIV-associated nephropathy with corticosteroids

Eustace, J. A., Nuermberger, E., Choi, M., Scheel, J., Moore, R. & Briggs, W. A., Jan 1 2000, In : Kidney International. 58, 3, p. 1253-1260 8 p.

Research output: Contribution to journalArticle

Open Access
109 Scopus citations

Glucosidase and mannosidase inhibitors mediate increased secretion of mutant α1 antitrypsin Z

Marcus, N. Y. & Perlmutter, D. H., Jan 21 2000, In : Journal of Biological Chemistry. 275, 3, p. 1987-1992 6 p.

Research output: Contribution to journalArticle

Open Access
66 Scopus citations

Liver injury in alpha1-antitrypsin deficiency

Perlmutter, D. H., Jan 1 2000, In : Clinics in Liver Disease. 4, 2, p. 387-408 22 p.

Research output: Contribution to journalArticle

25 Scopus citations

Relationship between lymphocyte and clinical steroid responsiveness in focal segmental glomerulosclerosis

Briggs, W. A., Gimenez, L. E., Samaniego-Picota, M., Choi, M. J., Nadasdy, T., Eustace, J. & Scheel, P. J., Jan 1 2000, In : Journal of clinical pharmacology. 40, 2, p. 115-123 9 p.

Research output: Contribution to journalArticle

11 Scopus citations
225 Scopus citations
61 Scopus citations

α1-antitrypsin deficiency-associated liver disease progresses slowly in some children

Volpert, D., Molleston, J. P. & Perlmutter, D. H., Sep 14 2000, In : Journal of pediatric gastroenterology and nutrition. 31, 3, p. 258-263 6 p.

Research output: Contribution to journalArticle

35 Scopus citations
1999

Anomalous development of the hepatobiliary system in the inv mouse

Mazziotti, M. V., Willis, L. K., Heuckeroth, R. O., LaRegina, M. C., Swanson, P. E., Overbeek, P. A. & Perlmutter, D. H., Aug 9 1999, In : Hepatology. 30, 2, p. 372-378 7 p.

Research output: Contribution to journalArticle

Open Access
95 Scopus citations

Chain length of the polylysine in receptor-targeted gene transfer complexes affects duration of reporter gene expression both in vitro and in vivo

Ziady, A. G., Ferkol, T., Dawson, D. V., Perlmutter, D. H. & Davis, P. B., Feb 19 1999, In : Journal of Biological Chemistry. 274, 8, p. 4908-4916 9 p.

Research output: Contribution to journalArticle

Open Access
67 Scopus citations

Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency

Schmidt, B. Z., Fowler, N. L., Hidvegi, T., Perlmutter, D. H. & Colten, H. R., Apr 23 1999, In : Journal of Biological Chemistry. 274, 17, p. 11782-11788 7 p.

Research output: Contribution to journalArticle

Open Access
35 Scopus citations

Primary gastric plasmacytoma: A rare cause of hypertrophic gastritis in an adolescent

Goyal, A., Langer, J. C., Zutter, M., Swanson, P., Kraus, M. D., Bartlett, N., Shackelford, G. D., Longtine, J. A. & Perlmutter, D. H., Oct 1 1999, In : Journal of pediatric gastroenterology and nutrition. 29, 4, p. 424-430 7 p.

Research output: Contribution to journalArticle

12 Scopus citations
33 Scopus citations
1998

Laparoscopic renal biopsy

Gimenez, L. F., Micali, S., Chen, R. N., Moore, R. G., Kavoussi, L. R. & Scheel, P. J., Jan 1 1998, In : Kidney International. 54, 2, p. 525-529 5 p.

Research output: Contribution to journalArticle

Open Access
58 Scopus citations
24 Scopus citations

Successful mycophenolate mofetil treatment of glomerular disease

Briggs, W. A., Choi, M. J. & Scheel, P. J., Feb 1998, In : American Journal of Kidney Diseases. 31, 2, p. 213-217+364-365

Research output: Contribution to journalArticle

180 Scopus citations
1997

Gene transfer into hepatoma cell lines via the serpin enzyme complex receptor

Ziady, A. G., Perales, J. C., Ferkol, T., Gerken, T., Beegen, H., Perlmutter, D. H. & Davis, P. B., Oct 13 1997, In : American Journal of Physiology - Gastrointestinal and Liver Physiology. 273, 2 36-2, p. G545-G552

Research output: Contribution to journalArticle

33 Scopus citations

Reconstitution of bile acid transport in a heterologous cell by cotransfection of transporters for bile acid uptake and efflux

Sippel, C. J., Dawson, P. A., Shen, T. & Perlmutter, D. H., Jul 18 1997, In : Journal of Biological Chemistry. 272, 29, p. 18290-18297 8 p.

Research output: Contribution to journalArticle

Open Access
21 Scopus citations
1996

Biliary atresia: Current concepts and research directions. Summary of a symposium

Balistreri, W. F., Grand, R., Hoofnagle, J. H., Suchy, F. J., Ryckman, F. C., Perlmutter, D. & Sokol, R. J., Jun 24 1996, In : Hepatology. 23, 6, p. 1682-1692 11 p.

Research output: Contribution to journalArticle

Open Access

Biliary atresia: Current concepts and research directions summary of a symposium

Balistreri, W. F., Grand, R., Hoofnagle, J. H., Suchy, F. J., Ryckman, F. C., Perlmutter, D. H. & Sokol, R. J., Jan 1 1996, In : Hepatology. 23, 6, p. 1682-1692 11 p.

Research output: Contribution to journalArticle

Open Access
264 Scopus citations

Degradation of a mutant secretory protein, α 1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity

Qu, D., Teckman, J. H., Omura, S. & Perlmutter, D. H., Sep 25 1996, In : Journal of Biological Chemistry. 271, 37, p. 22791-22795 5 p.

Research output: Contribution to journalArticle

Open Access
289 Scopus citations