Search Results - "Hale, Pamela"

Induced pluripotent stem cells model personalized variations in liver disease resulting from α1‐antitrypsin deficiency by Tafaleng, Edgar N., Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto‐Gutierrez, Alejandro, Feghali‐Bostwick, Carol A., Wilson, Andrew A., Kotton, Darrell N., Nagaya, Masaki, Strom, Stephen C., Roy‐Chowdhury, Jayanta, Stolz, Donna B., Perlmutter, David H., Fox, Ira J.

“…In the classical form of α1‐antitrypsin deficiency (ATD), aberrant intracellular accumulation of misfolded mutant α1‐antitrypsin Z (ATZ) in hepatocytes causes…”
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An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z by Wang, Yan, Cobanoglu, Murat C, Li, Jie, Hidvegi, Tunda, Hale, Pamela, Ewing, Michael, Chu, Andrew S, Gong, Zhenwei, Muzumdar, Radhika, Pak, Stephen C, Silverman, Gary A, Bahar, Ivet, Perlmutter, David H

“…The classical form of α1-antitrypsin deficiency (ATD) is characterized by intracellular accumulation of the misfolded variant α1-antitrypsin Z (ATZ) and severe…”
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Clathrin Pit-mediated Endocytosis of Neutrophil Elastase and Cathepsin G by Cancer Cells by Gregory, Alyssa D., Hale, Pamela, Perlmutter, David H., Houghton, A.McGarry

“…Neutrophil elastase (NE) is a neutrophil-derived serine proteinase with broad substrate specificity. We have recently demonstrated that NE is capable of…”
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Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency by Li, Jie, Pak, Stephen C, O'Reilly, Linda P, Benson, Joshua A, Wang, Yan, Hidvegi, Tunda, Hale, Pamela, Dippold, Christine, Ewing, Michael, Silverman, Gary A, Perlmutter, David H

“…The classical form of α1-antitrypsin deficiency (ATD) is associated with hepatic fibrosis and hepatocellular carcinoma. It is caused by the proteotoxic effect…”
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The Arg92Cys colipase polymorphism impairs function and secretion by increasing protein misfolding by Xiao, Xunjun, Ferguson, Michael R., Magee, Kelsey E., Hale, Pamela D., Wang, Yan, Lowe, Mark E.

“…Colipase is essential for efficient fat digestion. An arginine-to-cysteine polymorphism at position 92 of colipase (Arg92Cys) associates with an increased risk…”
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Autophagy-Enhancing Drug Promotes Degradation of Mutant α₁-Antitrypsin Z and Reduces Hepatic Fibrosis by Hidvegi, Tunda, Ewing, Michael, Hale, Pamela, Dippold, Christine, Beckett, Caroline, Kemp, Carolyn, Maurice, Nicholas, Mukherjee, Amitava, Goldbach, Christina, Watkins, Simon, Michalopoulos, George, Perlmutter, David H

“…In the classical form of α₁-antitrypsin (AT) deficiency, a point mutation in AT alters the folding of a liver-derived secretory glycoprotein and renders it…”
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An Autophagy-Enhancing Drug Promotes Degradation of Mutant α 1 -Antitrypsin Z and Reduces Hepatic Fibrosis by Hidvegi, Tunda, Ewing, Michael, Hale, Pamela, Dippold, Christine, Beckett, Caroline, Kemp, Carolyn, Maurice, Nicholas, Mukherjee, Amitava, Goldbach, Christina, Watkins, Simon, Michalopoulos, George, Perlmutter, David H.

“…The classical form of α 1 -antitrypsin (AT) deficiency is caused by a point mutation that alters the folding and causes intracellular aggregation of AT—an…”
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Regulation of PGC1α Downstream of the Insulin Signaling Pathway Plays a Role in the Hepatic Proteotoxicity of Mutant α1-Antitrypsin Deficiency Variant Z by Rudnick, David A., Huang, Jiansheng, Hidvegi, Tunda, Chu, Andrew S., Hale, Pamela, Munanairi, Admire, Dietzen, Dennis J., Cliften, Paul F., Tycksen, Eric, Lutkewitte, Andrew J., Finck, Brian N., Pak, Stephen C., Silverman, Gary A., Perlmutter, David H.

“…Insulin signaling is known to regulate essential proteostasis mechanisms. The analyses here examined effects of insulin signaling in the PiZ mouse model of…”
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Induced pluripotent stem cells model personalized variations in liver disease resulting from [alpha]1-antitrypsin deficiency by Tafaleng, Edgar N, Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto-Gutierrez, Alejandro, Feghali-Bostwick, Carol A, Wilson, Andrew A, Kotton, Darrell N, Nagaya, Masaki, Strom, Stephen C, Roy-Chowdhury, Jayanta, Stolz, Donna B, Perlmutter, David H, Fox, Ira J

“…In the classical form of [alpha]1-antitrypsin deficiency (ATD), aberrant intracellular accumulation of misfolded mutant [alpha]1-antitrypsin Z (ATZ) in…”
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Regulator of G Signaling 16 Is a Marker for the Distinct Endoplasmic Reticulum Stress State Associated with Aggregated Mutant α1-Antitrypsin Z in the Classical Form of α1-Antitrypsin Deficiency by Hidvegi, Tunda, Mirnics, Karoly, Hale, Pamela, Ewing, Michael, Beckett, Caroline, Perlmutter, David H.

“…In the classical form of α1-antitrypsin deficiency, a mutant protein accumulates in a polymerized form in the endoplasmic reticulum (ER) of liver cells causing…”
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An analog of glibenclamide selectively enhances autophagic degradation of misfolded [alpha]1-antitrypsin Z by Wang, Yan, Cobanoglu, Murat C, Li, Jie, Hidvegi, Tunda, Hale, Pamela, Ewing, Michael, Chu, Andrew S, Gong, Zhenwei, Muzumdar, Radhika, Pak, Stephen C, Silverman, Gary A, Bahar, Ivet, Perlmutter, David H

“…The classical form of [alpha]1-antitrypsin deficiency (ATD) is characterized by intracellular accumulation of the misfolded variant [alpha]1-antitrypsin Z…”
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A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency by O'Reilly, Linda P, Long, Olivia S, Cobanoglu, Murat C, Benson, Joshua A, Luke, Cliff J, Miedel, Mark T, Hale, Pamela, Perlmutter, David H, Bahar, Ivet, Silverman, Gary A, Pak, Stephen C

“…α1-Antitrypsin deficiency (ATD) is a common genetic disorder that can lead to end-stage liver and lung disease. Although liver transplantation remains the only…”
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Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy by Hidvegi, Tunda, Stolz, Donna B., Alcorn, John F., Yousem, Samuel A., Wang, Jieru, Leme, Adriana S., Houghton, A.McGarry, Hale, Pamela, Ewing, Michael, Cai, Houming, Garchar, Evelyn Akpadock, Pastore, Nunzia, Annunziata, Patrizia, Kaminski, Naftali, Pilewski, Joseph, Shapiro, Steven D., Pak, Stephen C., Silverman, Gary A., Brunetti-Pierri, Nicola, Perlmutter, David H.

“…Recent studies have shown that autophagy mitigates the pathological effects of proteinopathies in the liver, heart, and skeletal muscle but this has not been…”
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Clathrin-Mediated Endocytosis of Neutrophil Elastase and Cathepsin G by Lung Tumor Epithelial Cells by Gregory, Alyssa D., Hale, Pamela D., Perlmutter, David H., Shapiro, Steven D., Houghton, A. McGarry

“…Abstract 3269 Neutrophil elastase (NE) is an abundantly expressed enzyme with broad substrate specificity and the ability to regulate many components of the…”
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Induced pluripotent stem cells model personalized variations in liver disease due to α1-antitrypsin deficiency by Tafaleng, Edgar N., Chakraborty, Souvik, Han, Bing, Hale, Pamela, Wu, Wanquan, Soto-Gutierrez, Alejandro, Feghali-Bostwick, Carol A., Wilson, Andrew A., Kotton, Darrell N., Nagaya, Masaki, Strom, Stephen C., Chowdhury, Jayanta R., Stolz, Donna B., Perlmutter, David H., Fox, Ira J.

“…In the classical form of α 1 -antitrypsin deficiency (ATD), aberrant intracellular accumulation of misfolded mutant α 1 -antitrypsin Z (ATZ) in hepatocytes…”
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The Proteasome Participates in Degradation of Mutant α1-Antitrypsin Z in the Endoplasmic Reticulum of Hepatoma-derived Hepatocytes by Teckman, Jeffrey H., Burrows, Jon, Hidvegi, Tunde, Schmidt, Bela, Hale, Pamela D., Perlmutter, David H.

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COX-2 expression is induced by UVB exposure in human skin : Implications for the development of skin cancer by BUCKMAN, S. Y, GRESHAM, A, HALE, P, HRUZA, G, ANAST, J, MASFERRER, J, PENTLAND, A. P

“…Extensive documentation has validated the role of UV irradiation as a tumor initiator and promoter, inducing both squamous and basal cell carcinomas. Human…”
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The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes by Teckman, J H, Burrows, J, Hidvegi, T, Schmidt, B, Hale, P D, Perlmutter, D H

“…Because retention of mutant alpha(1)-antitrypsin (alpha(1)-AT) Z in the endoplasmic reticulum (ER) is associated with liver disease in alpha(1)-AT-deficient…”
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Improvement of Cortical Morphology in Infantile Hydrocephalic Animals after Ventriculoperitoneal Shunt Placement by Hale, Pamela M., McAllister, James P., Katz, Steven D., Wright, Lyn Carey, Lovely, Thomas J., Miller, David W., Wolfson, Barbara J., Salotto, Arnold G., Shroff, Deven V.

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A patient with pseudo-Addison's disease and falsely elevated thyroxine due to interference in serum cortisol and free thyroxine immunoassays by two different mechanisms by Saleem, Mohamed, Lewis, John G, Florkowski, Christopher M, Mulligan, Guy P, George, Peter M, Hale, Pamela

“…Interference in immunoassays is a widely recognized problem, which could potentially lead to unnecessary investigations and treatment. We describe a case where…”
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