David Perlmutter

2000

Chemical chaperones mediate increased secretion of mutant α1-antitrypsin (α1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency

Burrows, J. A. J., Willis, L. K. & Perlmutter, D. H., Feb 15 2000, In: Proceedings of the National Academy of Sciences of the United States of America. 97, 4, p. 1796-1801 6 p.

Research output: Contribution to journal › Article › peer-review

395 Scopus citations

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 journal › Article › peer-review

Open Access

70 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 journal › Article › peer-review

Open Access

71 Scopus citations

Liver injury in alpha₁-antitrypsin deficiency

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

Research output: Contribution to journal › Article › peer-review

28 Scopus citations

Retention of mutant α₁-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response

Teckman, J. H. & Perlmutter, D. H., 2000, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 279, 5 42-5, p. G961-G974

Research output: Contribution to journal › Article › peer-review

262 Scopus citations

Role of ubiquitin in proteasomal degradation of mutant α₁-antitrypsin Z in the endoplasmic reticulum

Teckman, J. H., Gilmore, R. & Perlmutter, D. H., Jan 2000, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 278, 1 41-1, p. G39-G48

Research output: Contribution to journal › Article › peer-review

67 Scopus citations

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

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

Research output: Contribution to journal › Article › peer-review

Open Access

43 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., 1999, In: Hepatology. 30, 2, p. 372-378 7 p.

Research output: Contribution to journal › Article › peer-review

Open Access

110 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 journal › Article › peer-review

Open Access

70 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 journal › Article › peer-review

Open Access

39 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 1999, In: Journal of pediatric gastroenterology and nutrition. 29, 4, p. 424-430 7 p.

Research output: Contribution to journal › Article › peer-review

Open Access

14 Scopus citations

Regulation of α₁-antitrypsin gene expression in human intestinal epithelial cell line Caco-2 by HNF-1α and HNF-4

Hu, C. & Perlmutter, D. H., May 1999, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 276, 5 39-5, p. G1181-G1194

Research output: Contribution to journal › Article › peer-review

51 Scopus citations

1998

Ligand substitution of receptor targeted DNA complexes affects gene transfer into hepatoma cells

Ziady, A. G., Ferkol, T., Gerken, T., Dawson, D. V., Perlmutter, D. H. & Davis, P. B., 1998, In: Gene therapy. 5, 12, p. 1685-1697 13 p.

Research output: Contribution to journal › Article › peer-review

32 Scopus citations

Resistance to the apoptotic effect of aggregated amyloid-β peptide in several different cell types including neuronal- and hepatoma-derived cell lines

Mazziotti, M. & Perlmutter, D. H., Jun 1 1998, In: Biochemical Journal. 332, 2, p. 517-524 8 p.

Research output: Contribution to journal › Article › peer-review

Open Access

24 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., 1997, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 273, 2 36-2, p. G545-G552

Research output: Contribution to journal › Article › peer-review

Open Access

36 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 journal › Article › peer-review

Open Access

22 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. H. & Sokol, R. J., 1996, In: Hepatology. 23, 6, p. 1682-1692 11 p.

Research output: Contribution to journal › Article › peer-review

313 Scopus citations

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 journal › Article › peer-review

Open Access

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., 1996, In: Journal of Biological Chemistry. 271, 37, p. 22791-22795 5 p.

Research output: Contribution to journal › Article › peer-review

Open Access

319 Scopus citations

Molecular pathogenesis of liver disease in α₁-antitrypsin deficiency

Teckman, J. H., Qu, D. & Perlmutter, D. H., 1996, In: Hepatology. 24, 6, p. 1504-1516 13 p.

Research output: Contribution to journal › Article › peer-review

110 Scopus citations

Regulation of sucrase-isomaltase gene expression in human intestinal epithelial cells by inflammatory cytokines

Ziambaras, T., Rubin, D. C. & Perlmutter, D. H., Jan 12 1996, In: Journal of Biological Chemistry. 271, 2, p. 1237-1242 6 p.

Research output: Contribution to journal › Article › peer-review

Open Access

55 Scopus citations

Site-directed mutagenesis within an ectoplasmic ATPase consensus sequence abrogates the cell aggregating properties of the rat liver canalicular bile acid transporter/ecto-ATPase/cell CAM 105 and carcinoembryonic antigen

Sippel, C. J., Shen, T. & Perlmutter, D. H., 1996, In: Journal of Biological Chemistry. 271, 51, p. 33095-33104 10 p.

Research output: Contribution to journal › Article › peer-review

Open Access

17 Scopus citations

The endoplasmic reticulum degradation pathway for mutant secretory proteins α1-antitrypsin Z and S is distinct from that for an unassembled membrane protein

Teckman, J. H. & Perlmutter, D. H., 1996, In: Journal of Biological Chemistry. 271, 22, p. 13215-13220 6 p.

Research output: Contribution to journal › Article › peer-review

Open Access

89 Scopus citations

The serpin-enzyme complex receptor recognizes soluble, nontoxic amyloid- β peptide but not aggregated, cytotoxic amyloid-β peptide

Boland, K., Behrens, M., Choi, D., Manias, K. & Perlmutter, D. H., 1996, In: Journal of Biological Chemistry. 271, 30, p. 18032-18044 13 p.

Research output: Contribution to journal › Article › peer-review

Open Access

82 Scopus citations

1995

Cellular expression of the C5a anaphylatoxin receptor (C5aR): Demonstration of C5aR on nonmyeloid cells of the liver and lung

Haviland, D. L., McCoy, R. L., Whitehead, W. T., Akama, H., Molmenti, E. P., Brown, A., Haviland, J. C., Parks, W. C., Perlmutter, D. H. & Wetsel, R. A., Feb 15 1995, In: Journal of Immunology. 154, 4, p. 1861-1869 9 p.

Research output: Contribution to journal › Article › peer-review

220 Scopus citations

N-formylpeptide and complement C5a receptors are expressed in liver cells and mediate hepatic acute phase gene regulation

McCoy, R., Haviland, D. L., Molmenti, E. P., Ziambaras, T., Wetsel, R. A. & Perlmutter, D. H., Jul 1 1995, In: Journal of Experimental Medicine. 182, 1, p. 207-217 11 p.

Research output: Contribution to journal › Article › peer-review

Open Access

107 Scopus citations

Specificity in recognition of amyloid-β peptide by the serpin-enzyme complex receptor in hepatoma cells and neuronal cells

Boland, K., Manias, K. & Perlmutter, D. H., Nov 24 1995, In: Journal of Biological Chemistry. 270, 47, p. 28022-28028 7 p.

Research output: Contribution to journal › Article › peer-review

Open Access

43 Scopus citations

1994

A lag in intracellular degradation of mutant α₁-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ α₁-antitrypsin deficiency

Wu, Y., Whitman, I., Molmenti, E., Moore, K., Hippenmeyer, P. & Perlmutter, D. H., Sep 13 1994, In: Proceedings of the National Academy of Sciences of the United States of America. 91, 19, p. 9014-9018 5 p.

Research output: Contribution to journal › Article › peer-review

240 Scopus citations

Bile acid efflux mediated by the rat liver canalicular bile acid transport/ecto-ATPase protein requires serine 503 phosphorylation and is regulated by tyrosine 488 phosphorylation

Sippel, C. J., Fallon, R. J. & Perlmutter, D. H., Jul 29 1994, In: Journal of Biological Chemistry. 269, 30, p. 19539-19545 7 p.

Research output: Contribution to journal › Article › peer-review

49 Scopus citations

Bile Acid Transport by the Rat Liver Canalicular Bile Acid Transport/Ecto-ATPase Protein Is Dependent on ATP but Not on Its Own Ecto-ATPase Activity

Sippel, C. J., McCollum, M. J. & Perlmutter, D. H., Jan 28 1994, In: Journal of Biological Chemistry. 269, 4, p. 2820-2826 7 p.

Research output: Contribution to journal › Article › peer-review

46 Scopus citations

1993

Cell-specific expression of α₁-antitrypsin in human intestinal epithelium

Molmenti, E. P., Perlmutter, D. H. & Rubin, D. C., 1993, In: Journal of Clinical Investigation. 92, 4, p. 2022-2034 13 p.

Research output: Contribution to journal › Article › peer-review

Open Access

117 Scopus citations

Cross-competition for binding of α₁-antitrypsin (α₁ AT)-elastase complexes to the serpin-enzyme complex receptor by other serpin-enzyme complexes and by proteolytically modified α₁ AT

Joslin, G., Wittwer, A., Adams, S., Tollefsen, D. M., August, A. & Perlmutter, D. H., Jan 25 1993, In: Journal of Biological Chemistry. 268, 3, p. 1886-1893 8 p.

Research output: Contribution to journal › Article › peer-review

63 Scopus citations

Evidence for an acute phase response in human intestinal epithelial cells

Molmenti, E. P., Ziambaras, T. & Perlmutter, D. H., Jul 5 1993, In: Journal of Biological Chemistry. 268, 19, p. 14116-14124 9 p.

Research output: Contribution to journal › Article › peer-review

225 Scopus citations

The rat liver ecto-ATPase is also a canalicular bile acid transport protein

Sippel, C. J., Suchy, F. J., Ananthanarayanan, M. & Perlmutter, D. H., Jan 25 1993, In: Journal of Biological Chemistry. 268, 3, p. 2083-2091 9 p.

Research output: Contribution to journal › Article › peer-review

117 Scopus citations

1992

Constitutive and modulated expression of the human α₁ antitrypsin gene. Different transcriptional initiation sites used in three different cell types

Hafeez, W., Ciliberto, G. & Perlmutter, D. H., 1992, In: Journal of Clinical Investigation. 89, 4, p. 1214-1222 9 p.

Research output: Contribution to journal › Article › peer-review

Open Access

64 Scopus citations

The serpin-enzyme complex (SEC) receptor mediates the neutrophil chemotactic effect of α-₁ antitrypsin-elastase complexes and amyloid-β peptide

Joslin, G., Griffin, G. L., August, A. M., Adams, S., Fallen, R. J., Senior, R. M. & Perlmutter, D. H., 1992, In: Journal of Clinical Investigation. 90, 3, p. 1150-1154 5 p.

Research output: Contribution to journal › Article › peer-review

Open Access

73 Scopus citations

1991

Amyloid-β peptide, substance P, and bombesin bind to the serpin-enzyme complex receptor

Joslin, G., Krause, J. E., Hershey, A. D., Adams, S. P., Fallon, R. J. & Perlmutter, D. H., 1991, In: Journal of Biological Chemistry. 266, 32, p. 21897-21902 6 p.

Research output: Contribution to journal › Article › peer-review

106 Scopus citations

Effect of pseudomonas elastase on human mononuclear phagocyte α₁-antitrypsin expression

Barbey-Morel, C. & Perlmutter, D. H., Feb 1991, In: Pediatric research. 29, 2, p. 133-140 8 p.

Research output: Contribution to journal › Article › peer-review

Open Access

14 Scopus citations

Expression of stress proteins in human mononuclear phagocytes

Joslin, G., Hafeez, W. & Perlmutter, D. H., Sep 1 1991, In: Journal of Immunology. 147, 5, p. 1614-1620 7 p.

Research output: Contribution to journal › Article › peer-review

17 Scopus citations

The cellular basis for liver injury in α₁‐antitrypsin deficiency

Perlmutter, D. H., Jan 1991, In: Hepatology. 13, 1, p. 172-185 14 p.

Research output: Contribution to journal › Article › peer-review

Open Access

68 Scopus citations

The SEC receptor recognizes a pentapeptide neodomain of α₁-antitrypsin-protease complexes

Joslin, G., Fallon, R. J., Bullock, J., Adams, S. P. & Perlmutter, D. H., 1991, In: Journal of Biological Chemistry. 266, 17, p. 11282-11288 7 p.

Research output: Contribution to journal › Article › peer-review

107 Scopus citations

1990

Conference summary: Mechanisms and management of pediatric hepatobiliary disease

Balistreri, W. F., Boyer, J. L., Carpenter, G., Desmet, V. J., Fausto, N., Goldfischer, S. L., Horwitz, M. S., Hutton, J. J., Javitt, N. B., Lazarow, P. B., Lilly, J. R., McDonagh, A. F., Moser, H. W., Perlmutter, D. H., Schwartz, A. L., Shafritz, D. A. & Zuckerman, A. J., Jan 1990, In: Journal of pediatric gastroenterology and nutrition. 10, 1, p. 138-147 10 p.

Research output: Contribution to journal › Article › peer-review

3 Scopus citations

Counterregulatory effects of interferon-γ and endotoxin on expression of the human C4 genes

Kulics, J., Colten, H. R. & Perlmutter, D. H., Mar 1990, In: Journal of Clinical Investigation. 85, 3, p. 943-949 7 p.

Research output: Contribution to journal › Article › peer-review

44 Scopus citations

Endocytosis and degradation of α₁-Antitrypsin-protease complexes is mediated by the Serpin-Enzyme Complex (SEC) receptor

Perlmutter, D. H., Joslin, G., Nelson, P., Schasteen, C., Adams, S. P. & Fallon, R. J., Oct 5 1990, In: Journal of Biological Chemistry. 265, 28, p. 16713-16716 4 p.

Research output: Contribution to journal › Article › peer-review

115 Scopus citations

Identification of a serpin-enzyme complex receptor on human hepatoma cells and human monocytes

Perlmutter, D. H., Glover, G. I., Rivetna, M., Schasteen, C. S. & Fallon, R. J., 1990, In: Proceedings of the National Academy of Sciences of the United States of America. 87, 10, p. 3753-3757 5 p.

Research output: Contribution to journal › Article › peer-review

Open Access

176 Scopus citations

1989

A cytokine-selective defect in interleukin-1β-mediated acute phase gene expression in a subclone of the human hepatoma cell line (HepG2)

Perlmutter, D. H., Colten, H. R., Adams, S. P., May, L. T., Sehgal, P. B. & Fallon, R. J., 1989, In: Journal of Biological Chemistry. 264, 13, p. 7669-7674 6 p.

Research output: Contribution to journal › Article › peer-review

17 Scopus citations

IFNβ2/IL‐6 Is One of Several Cytokines That Modulate Acute Phase Gene Expression in Human Hepatocytes and Human Macrophages

PERLMUTTER, D. H., Jun 1989, In: Annals of the New York Academy of Sciences. 557, 1, p. 332-342 11 p.

Research output: Contribution to journal › Article › peer-review

9 Scopus citations

Interferon β2/interleukin 6 modulates synthesis of α₁-antitrypsin in human mononuclear phagocytes and in human hepatoma cells

Perlmutter, D. H., May, L. T. & Sehgal, P. B., 1989, In: Journal of Clinical Investigation. 84, 1, p. 138-144 7 p.

Research output: Contribution to journal › Article › peer-review

Open Access

114 Scopus citations

Intestinal alkaline phosphatase is secreted bidirectionally from villous enterocytes

Sussman, N. L., Eliakim, R., Rubin, D., Perlmutter, D. H., DeSchryver-Kecskemeti, K. & Alpers, D. H., 1989, In: American Journal of Physiology - Gastrointestinal and Liver Physiology. 257, 1, p. 20/1

Research output: Contribution to journal › Article › peer-review

56 Scopus citations

Synthesis of stress proteins is increased in individuals with homozygous PiZZ α₁-antitrypsin deficiency and liver disease

Perlmutter, D. H., Schlesinger, M. J., Pierce, J. A., Punsal, P. I. & Schwartz, A. L., 1989, In: Journal of Clinical Investigation. 84, 5, p. 1555-1561 7 p.

Research output: Contribution to journal › Article › peer-review

Open Access

47 Scopus citations

David Perlmutter

Research output

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