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David Perlmutter

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Cristy L Gelling; David H Perlmutter; Ian W Dawes; Edward A Fisher; Jeffrey L Brodsky
The endosomal protein-sorting receptor sortilin has a role in trafficking α-1 antitrypsin.
Nicholas Maurice; David H Perlmutter
Novel treatment strategies for liver disease due to α1-antitrypsin deficiency.
Jianqiang Ding; Govardhana R Yannam; Namita Roy-Chowdhury; Tunda Hidvegi; Hesham Basma; Stephen I Rennard; Ronald J Wong; Yesim Avsar; Chandan Guha; David H Perlmutter; Ira J Fox; Jayanta Roy-Chowdhury
Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes.

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All Publications

2012: Cristy L Gelling; David H Perlmutter; Ian W Dawes; Edward A Fisher; Jeffrey L Brodsky
The endosomal protein-sorting receptor sortilin has a role in trafficking α-1 antitrypsin.
Genetics 2012;192(3):889-903.
2012: Nicholas Maurice; David H Perlmutter
Novel treatment strategies for liver disease due to α1-antitrypsin deficiency.
Clinical and translational science 2012;5(3):289-94.
2011: Jianqiang Ding; Govardhana R Yannam; Namita Roy-Chowdhury; Tunda Hidvegi; Hesham Basma; Stephen I Rennard; Ronald J Wong; Yesim Avsar; Chandan Guha; David H Perlmutter; Ira J Fox; Jayanta Roy-Chowdhury
Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes.
The Journal of clinical investigation 2011;121(5):1930-4.
2011: David H Perlmutter; Gary A Silverman
Hepatic fibrosis and carcinogenesis in α1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders.
Cold Spring Harbor perspectives in biology 2011;3(3):.
2011: Tunda Hidvegi; Carolyn Kemp; Amitava Mukherjee; Michael Ewing; David H Perlmutter
The role of autophagy in alpha-1-antitrypsin deficiency.
Methods in enzymology 2011;499():33-54.
2010: Tunda Hidvegi; Michael Ewing; Pamela Hale; CS Dippold; Caroline Beckett; Carolyn Kemp; Nicholas Maurice; Amitava Mukherjee; Christina Goldbach; simon simon watkins; George Michalopoulos; David H Perlmutter
An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.
Science (New York, N.Y.) 2010;329(5988):229-32.
2007: Craig M Scott; Kristina B Kruse; Bela Z Schmidt; David H Perlmutter; Ardythe A McCracken; Jeffrey L Brodsky
ADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly.
Molecular biology of the cell 2007;18(10):3776-87.
2007: Tunda Hidvegi; Károly Mirnics; Pamela Hale; Michael Ewing; Caroline Beckett; David H Perlmutter
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.
The Journal of biological chemistry 2007;282(38):27769-80.
2007: David H Perlmutter; Jeffrey L Brodsky; William F Balistreri; Bruce C Trapnell
Molecular pathogenesis of alpha-1-antitrypsin deficiency-associated liver disease: a meeting review.
Hepatology (Baltimore, Md.) 2007;45(5):1313-23.
2006: David H Perlmutter
The role of autophagy in alpha-1-antitrypsin deficiency: a specific cellular response in genetic diseases associated with aggregation-prone proteins.
Autophagy 2006;2(4):258-63.
2006: David H Perlmutter
Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.
Pediatric research 2006;60(2):233-8.
2006: Takahiro Kamimoto; Shisako Shoji; Tunda Hidvegi; Noboru Mizushima; Kyohei Umebayashi; David H Perlmutter; Tamotsu Yoshimori
Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity.
The Journal of biological chemistry 2006;281(7):4467-76.
2005: Tunda Hidvegi; Bela Z Schmidt; Pamela Hale; David H Perlmutter
Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.
The Journal of biological chemistry 2005;280(47):39002-15.
2005: David A Rudnick; David H Perlmutter
Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease.
Hepatology (Baltimore, Md.) 2005;42(3):514-21.
2005: Bela Z Schmidt; David H Perlmutter
Grp78, Grp94, and Grp170 interact with alpha1-antitrypsin mutants that are retained in the endoplasmic reticulum.
American journal of physiology. Gastrointestinal and liver physiology 2005;289(3):G444-55.
2004: David H Perlmutter
Alpha-1-antitrypsin deficiency: diagnosis and treatment.
Clinics in liver disease 2004;8(4):839-59, viii-ix.
2004: Jeffrey H Teckman; Jae-Koo An; Keith Blomenkamp; Bela Schmidt; David H Perlmutter
Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.
American journal of physiology. Gastrointestinal and liver physiology 2004;286(5):G851-62.
2004: David A Rudnick; Yunjun Liao; Jae-Koo An; Louis J Muglia; David H Perlmutter; Jeffrey H Teckman
Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency.
Hepatology (Baltimore, Md.) 2004;39(4):1048-55.
2003: David H Perlmutter
Alpha1-antitrypsin deficiency: liver disease associated with retention of a mutant secretory glycoprotein in the endoplasmic reticulum.
Methods in molecular biology (Clifton, N.J.) 2003;232():39-56.
2002: David H Perlmutter
Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury.
The Journal of clinical investigation 2002;110(11):1579-83.
2002: David H Perlmutter
Chemical chaperones: a pharmacological strategy for disorders of protein folding and trafficking.
Pediatric research 2002;52(6):832-6.
2002: Jeffrey H Teckman; Jae-Koo An; Scott Loethen; David H Perlmutter
Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy.
American journal of physiology. Gastrointestinal and liver physiology 2002;283(5):G1156-65.
2002: David H Perlmutter; Ross W Shepherd
Extrahepatic biliary atresia: a disease or a phenotype?
Hepatology (Baltimore, Md.) 2002;35(6):1297-304.
2002: Chaobin Hu; David H Perlmutter
Cell-specific involvement of HNF-1beta in alpha(1)-antitrypsin gene expression in human respiratory epithelial cells.
American journal of physiology. Lung cellular and molecular physiology 2002;282(4):L757-65.
2002: David H Perlmutter
Metabolic liver disease.
Journal of pediatric gastroenterology and nutrition 2002;35 Suppl 1():S24-8.
2001: Jeffrey H Teckman; Jon Burrows; Tunda Hidvegi; B Schmidt; Pamela Hale; David H Perlmutter
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.
The Journal of biological chemistry 2001;276(48):44865-72.
2001: L Lin; B Schmidt; Jeffrey H Teckman; David H Perlmutter
A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum.
The Journal of biological chemistry 2001;276(36):33893-8.
2001: David A Rudnick; David H Perlmutter; Louis J Muglia
Prostaglandins are required for CREB activation and cellular proliferation during liver regeneration.
Proceedings of the National Academy of Sciences of the United States of America 2001;98(15):8885-90.
2001: D Volpert; F White; M J Finegold; Jean P Molleston; Michael R DeBaun; David H Perlmutter
Outcome of early hepatic portoenterostomy for biliary atresia.
Journal of pediatric gastroenterology and nutrition 2001;32(3):265-9.
2000: Jeffrey H Teckman; David H Perlmutter
Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response.
American journal of physiology. Gastrointestinal and liver physiology 2000;279(5):G961-74.
2000: D Volpert; Jean P Molleston; David H Perlmutter
Alpha1-antitrypsin deficiency-associated liver disease progresses slowly in some children.
Journal of pediatric gastroenterology and nutrition 2000;31(3):258-63.
2000: Jeffrey H Teckman; R Gilmore; David H Perlmutter
Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum.
American journal of physiology. Gastrointestinal and liver physiology 2000;278(1):G39-48.
1999: David H Perlmutter
Misfolded proteins in the endoplasmic reticulum.
Laboratory investigation; a journal of technical methods and pathology 1999;79(6):623-38.
1999: C Hu; David H Perlmutter
Regulation of alpha1-antitrypsin gene expression in human intestinal epithelial cell line caco-2 by HNF-1alpha and HNF-4.
The American journal of physiology 1999;276(5 Pt 1):G1181-94.
1999: Bela Z Schmidt; N L Fowler; Tunda Hidvegi; David H Perlmutter; HR Colten
Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency.
The Journal of biological chemistry 1999;274(17):11782-8.
1998: N Marcus; Jeffrey H Teckman; David H Perlmutter
Alpha1-antitrypsin deficiency: from genotype to childhood disease.
Journal of pediatric gastroenterology and nutrition 1998;27(1):65-74.
1998: David H Perlmutter
Alpha-1-antitrypsin deficiency.
Seminars in liver disease 1998;18(3):217-25.
1997: C J Sippel; P A Dawson; T Shen; David H Perlmutter
Reconstitution of bile acid transport in a heterologous cell by cotransfection of transporters for bile acid uptake and efflux.
The Journal of biological chemistry 1997;272(29):18290-7.
1997: D Qu; Jeffrey H Teckman; David H Perlmutter
Review: alpha 1-antitrypsin deficiency associated liver disease.
Journal of gastroenterology and hepatology 1997;12(5):404-16.
1996: C J Sippel; T Shen; David H Perlmutter
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.
The Journal of biological chemistry 1996;271(51):33095-104.
1996: Jeffrey H Teckman; D Qu; David H Perlmutter
Molecular pathogenesis of liver disease in alpha1-antitrypsin deficiency.
Hepatology (Baltimore, Md.) 1996;24(6):1504-16.
1996: D Qu; Jeffrey H Teckman; S Omura; David H Perlmutter
Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity.
The Journal of biological chemistry 1996;271(37):22791-5.
1996: K Boland; M Behrens; D Choi; K Manias; David H Perlmutter
The serpin-enzyme complex receptor recognizes soluble, nontoxic amyloid-beta peptide but not aggregated, cytotoxic amyloid-beta peptide.
The Journal of biological chemistry 1996;271(30):18032-44.
1996: Jeffrey H Teckman; David H Perlmutter
The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein.
The Journal of biological chemistry 1996;271(22):13215-20.
1996: T Ziambaras; Deborah C Rubin; David H Perlmutter
Regulation of sucrase-isomaltase gene expression in human intestinal epithelial cells by inflammatory cytokines.
The Journal of biological chemistry 1996;271(2):1237-42.
1995: David H Perlmutter
Society for Pediatric Research presidential address 1995: marrying our medicine to biology.
Pediatric research 1995;38(6):830-4.
1995: R McCoy; D L Haviland; Ernesto Molmenti; T Ziambaras; Rick A Wetsel; David H Perlmutter
N-formylpeptide and complement C5a receptors are expressed in liver cells and mediate hepatic acute phase gene regulation.
The Journal of experimental medicine 1995;182(1):207-17.
1995: Jeffrey H Teckman; David H Perlmutter
Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.
Gastroenterology 1995;108(4):1263-79.
1995: D L Haviland; R L McCoy; W T Whitehead; H Akama; Ernesto Molmenti; A Brown; J C Haviland; W C Parks; David H Perlmutter; Rick A Wetsel
Cellular expression of the C5a anaphylatoxin receptor (C5aR): demonstration of C5aR on nonmyeloid cells of the liver and lung.
Journal of immunology (Baltimore, Md. : 1950) 1995;154(4):1861-9.
1994: Y Wu; I Whitman; Ernesto Molmenti; K Moore; P Hippenmeyer; David H Perlmutter
A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.
Proceedings of the National Academy of Sciences of the United States of America 1994;91(19):9014-8.
1994: David H Perlmutter
The SEC receptor: a possible link between neonatal hepatitis in alpha 1-antitrypsin deficiency and Alzheimer's disease.
Pediatric research 1994;36(3):271-7.
1994: C J Sippel; robert joseph fallon; David H Perlmutter
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.
The Journal of biological chemistry 1994;269(30):19539-45.
1994: David H Perlmutter
Metabolic liver disease in childhood.
Seminars in gastrointestinal disease 1994;5(2):54-64.
1994: C J Sippel; M J McCollum; David H Perlmutter
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.
The Journal of biological chemistry 1994;269(4):2820-6.
1993: Ernesto Molmenti; David H Perlmutter; D C Rubin
Cell-specific expression of alpha 1-antitrypsin in human intestinal epithelium.
The Journal of clinical investigation 1993;92(4):2022-34.
1993: Ernesto Molmenti; T Ziambaras; David H Perlmutter
Evidence for an acute phase response in human intestinal epithelial cells.
The Journal of biological chemistry 1993;268(19):14116-24.
1993: C J Sippel; Frederick J Suchy; Meenakshisundaram Ananthanarayanan; David H Perlmutter
The rat liver ecto-ATPase is also a canalicular bile acid transport protein.
The Journal of biological chemistry 1993;268(3):2083-91.
1993: G Joslin; A Wittwer; S Adams; D M Tollefsen; A August; David H Perlmutter
Cross-competition for binding of alpha 1-antitrypsin (alpha 1 AT)-elastase complexes to the serpin-enzyme complex receptor by other serpin-enzyme complexes and by proteolytically modified alpha 1 AT.
The Journal of biological chemistry 1993;268(3):1886-93.
1992: G Joslin; G L Griffin; A M August; S Adams; robert joseph fallon; R M Senior; David H Perlmutter
The serpin-enzyme complex (SEC) receptor mediates the neutrophil chemotactic effect of alpha-1 antitrypsin-elastase complexes and amyloid-beta peptide.
The Journal of clinical investigation 1992;90(3):1150-4.
1992: Waseem Hafeez; G Ciliberto; David H Perlmutter
Constitutive and modulated expression of the human alpha 1 antitrypsin gene. Different transcriptional initiation sites used in three different cell types.
The Journal of clinical investigation 1992;89(4):1214-22.
1991: G Joslin; J E Krause; A D Hershey; S P Adams; robert joseph fallon; David H Perlmutter
Amyloid-beta peptide, substance P, and bombesin bind to the serpin-enzyme complex receptor.
The Journal of biological chemistry 1991;266(32):21897-902.
1991: G Joslin; Waseem Hafeez; David H Perlmutter
Expression of stress proteins in human mononuclear phagocytes.
Journal of immunology (Baltimore, Md. : 1950) 1991;147(5):1614-20.
1991: G Joslin; robert joseph fallon; J Bullock; S P Adams; David H Perlmutter
The SEC receptor recognizes a pentapeptide neodomain of alpha 1-antitrypsin-protease complexes.
The Journal of biological chemistry 1991;266(17):11282-8.
1991: C Barbey-Morel; David H Perlmutter
Effect of pseudomonas elastase on human mononuclear phagocyte alpha 1-antitrypsin expression.
Pediatric research 1991;29(2):133-40.
1991: David H Perlmutter
The cellular basis for liver injury in alpha 1-antitrypsin deficiency.
Hepatology (Baltimore, Md.) 1991;13(1):172-85.
1990: David H Perlmutter; G Joslin; P Nelson; C Schasteen; S P Adams; robert joseph fallon
Endocytosis and degradation of alpha 1-antitrypsin-protease complexes is mediated by the serpin-enzyme complex (SEC) receptor.
The Journal of biological chemistry 1990;265(28):16713-6.
1990: David H Perlmutter; G I Glover; M Rivetna; C S Schasteen; robert joseph fallon
Identification of a serpin-enzyme complex receptor on human hepatoma cells and human monocytes.
Proceedings of the National Academy of Sciences of the United States of America 1990;87(10):3753-7.
1990: J Kulics; HR Colten; David H Perlmutter
Counterregulatory effects of interferon-gamma and endotoxin on expression of the human C4 genes.
The Journal of clinical investigation 1990;85(3):943-9.
1989: David H Perlmutter; M J Schlesinger; J A Pierce; P I Punsal; Alan L Schwartz
Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease.
The Journal of clinical investigation 1989;84(5):1555-61.
1989: David H Perlmutter; J A Pierce
The alpha 1-antitrypsin gene and emphysema.
The American journal of physiology 1989;257(4 Pt 1):L147-62.
1989: David H Perlmutter; L T May; P B Sehgal
Interferon beta 2/interleukin 6 modulates synthesis of alpha 1-antitrypsin in human mononuclear phagocytes and in human hepatoma cells.
The Journal of clinical investigation 1989;84(1):138-44.
1989: David H Perlmutter; J D Daniels; H S Auerbach; K De Schryver-Kecskemeti; H S Winter; D H Alpers
The alpha 1-antitrypsin gene is expressed in a human intestinal epithelial cell line.
The Journal of biological chemistry 1989;264(16):9485-90.
1989: David H Perlmutter; HR Colten; S P Adams; L T May; P B Sehgal; robert joseph fallon
A cytokine-selective defect in interleukin-1 beta-mediated acute phase gene expression in a subclone of the human hepatoma cell line (HEPG2).
The Journal of biological chemistry 1989;264(13):7669-74.
1989: David H Perlmutter; HR Colten
Molecular basis of complement deficiencies.
Immunodeficiency reviews 1989;1(2):105-33.
1988: David H Perlmutter; P I Punsal
Distinct and additive effects of elastase and endotoxin on expression of alpha 1 proteinase inhibitor in mononuclear phagocytes.
The Journal of biological chemistry 1988;263(31):16499-503.
1988: David H Perlmutter; J Travis; P I Punsal
Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency.
The Journal of clinical investigation 1988;81(6):1774-80.
1988: David H Perlmutter; M J Schlesinger; J A Pierce; E J Campbell; R J Rothbaum; Alan L Schwartz
Induction of the stress response in alpha 1-antitrypsin deficiency.
Transactions of the Association of American Physicians 1988;101():33-41.
1987: C Barbey-Morel; J A Pierce; E J Campbell; David H Perlmutter
Lipopolysaccharide modulates the expression of alpha 1 proteinase inhibitor and other serine proteinase inhibitors in human monocytes and macrophages.
The Journal of experimental medicine 1987;166(4):1041-54.
1987: J W Mier; C A Dinarello; M B Atkins; P I Punsal; David H Perlmutter
Regulation of hepatic acute phase protein synthesis by products of interleukin 2 (IL 2)-stimulated human peripheral blood mononuclear cells.
Journal of immunology (Baltimore, Md. : 1950) 1987;139(4):1268-72.
1987: David H Perlmutter; HR Colten
Structure and expression of the complement genes.
Pharmacology & therapeutics 1987;34(2):247-70.
1986: David H Perlmutter; C A Dinarello; P I Punsal; H R Colten
Cachectin/tumor necrosis factor regulates hepatic acute-phase gene expression.
The Journal of clinical investigation 1986;78(5):1349-54.