Search Results - "Starost, Matthew F"

Accumulation of the Inner Nuclear Envelope Protein Sun1 Is Pathogenic in Progeric and Dystrophic Laminopathies by Chen, Chia-Yen, Chi, Ya-Hui, Mutalif, Rafidah Abdul, Starost, Matthew F., Myers, Timothy G., Anderson, Stasia A., Stewart, Colin L., Jeang, Kuan-Teh

“…Human LMNA gene mutations result in laminopathies that include Emery-Dreifuss muscular dystrophy (AD-EDMD) and Hutchinson-Gilford progeria, the premature aging…”
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Intranasal or airborne transmission-mediated delivery of an attenuated SARS-CoV-2 protects Syrian hamsters against new variants by Stauft, Charles B., Selvaraj, Prabhuanand, D’Agnillo, Felice, Meseda, Clement A., Liu, Shufeng, Pedro, Cyntia L., Sangare, Kotou, Lien, Christopher Z., Weir, Jerry P., Starost, Matthew F., Wang, Tony T.

“…Detection of secretory antibodies in the airway is highly desirable when evaluating mucosal protection by vaccines against a respiratory virus, such as the…”
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Intranasal delivery of a rationally attenuated SARS-CoV-2 is immunogenic and protective in Syrian hamsters by Liu, Shufeng, Stauft, Charles B., Selvaraj, Prabhuanand, Chandrasekaran, Prabha, D’Agnillo, Felice, Chou, Chao-Kai, Wu, Wells W., Lien, Christopher Z., Meseda, Clement A., Pedro, Cyntia L., Starost, Matthew F., Weir, Jerry P., Wang, Tony T.

“…Few live attenuated severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines are in pre-clinical or clinical development. We seek to attenuate…”
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The phenotypic landscape of a Tbc1d24 mutant mouse includes convulsive seizures resembling human early infantile epileptic encephalopathy by Tona, Risa, Chen, Wenqian, Nakano, Yoko, Reyes, Laura D, Petralia, Ronald S, Wang, Ya-Xian, Starost, Matthew F, Wafa, Talah T, Morell, Robert J, Cravedi, Kevin D, du Hoffmann, Johann, Miyoshi, Takushi, Munasinghe, Jeeva P, Fitzgerald, Tracy S, Chudasama, Yogita, Omori, Koichi, Pierpaoli, Carlo, Banfi, Botond, Dong, Lijin, Belyantseva, Inna A, Friedman, Thomas B

“…Abstract Epilepsy, deafness, onychodystrophy, osteodystrophy and intellectual disability are associated with a spectrum of mutations of human TBC1D24. The…”
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A Mouse Homolog of a Human TP53 Germline Mutation Reveals a Lipolytic Activity of p53 by Kang, Ju-Gyeong, Lago, Cory U., Lee, Ji-Eun, Park, Ji-Hoon, Donnelly, Matthew P., Starost, Matthew F., Liu, Chengyu, Kwon, Jaeyul, Noguchi, Audrey C., Ge, Kai, Wang, Ping-yuan, Hwang, Paul M.

“…The physiological effects of the many germline mutations of TP53, encoding the tumor suppressor protein p53, are poorly understood. Here we report generating a…”
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Galnt1 is required for normal heart valve development and cardiac function by Tian, E, Stevens, Sharon R, Guan, Yu, Springer, Danielle A, Anderson, Stasia A, Starost, Matthew F, Patel, Vyomesh, Ten Hagen, Kelly G, Tabak, Lawrence A

“…Congenital heart valve defects in humans occur in approximately 2% of live births and are a major source of compromised cardiac function. In this study we…”
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Deafness and Stria Vascularis Defects in S1P2 Receptor-null Mice by Kono, Mari, Belyantseva, Inna A., Skoura, Athanasia, Frolenkov, Gregory I., Starost, Matthew F., Dreier, Jennifer L., Lidington, Darcy, Bolz, Steffen-Sebastian, Friedman, Thomas B., Hla, Timothy, Proia, Richard L.

“…The S1P2 receptor is a member of a family of G protein-coupled receptors that bind the extracellular sphingolipid metabolite sphingosine 1-phosphate with high…”
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Mutation in the key enzyme of sialic acid biosynthesis causes severe glomerular proteinuria and is rescued by N-acetylmannosamine by Galeano, Belinda, Klootwijk, Riko, Manoli, Irini, Sun, MaoSen, Ciccone, Carla, Darvish, Daniel, Starost, Matthew F, Zerfas, Patricia M, Hoffmann, Victoria J, Hoogstraten-Miller, Shelley, Krasnewich, Donna M, Gahl, William A, Huizing, Marjan

“…Mutations in the key enzyme of sialic acid biosynthesis, uridine diphospho-N-acetylglucosamine 2-epimerase/N-acetylmannosamine (ManNAc) kinase (GNE/MNK),…”
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TEL-AML1 Transgenic Zebrafish Model of Precursor B Cell Acute Lymphoblastic Leukemia by Sabaawy, Hatem E., Azuma, Mizuki, Embree, Lisa J., Tsai, Huai-Jen, Starost, Matthew F., Hickstein, Dennis D.

“…Acute lymphoblastic leukemia (ALL) is a clonal disease that evolves through the accrual of genetic rearrangements and/or mutations within the dominant clone…”
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SARS-CoV-2 B.1.1.7 Infection of Syrian Hamster Does Not Cause More Severe Disease, and Naturally Acquired Immunity Confers Protection by Nuñez, Ivette A, Lien, Christopher Z, Selvaraj, Prabhuanand, Stauft, Charles B, Liu, Shufeng, Starost, Matthew F, Wang, Tony T

“…Epidemiological studies have revealed the emergence of multiple severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) variants of concern (VOC),…”
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Light‐activatable minimally invasive ethyl cellulose ethanol ablation: Biodistribution and potential applications by Yang, Jeffrey, Ma, Chen‐Hua, Quinlan, John A., McNaughton, Kathryn, Lee, Taya, Shin, Peter, Hauser, Tessa, Kaluzienski, Michele L., Vig, Shruti, Quang, Tri T., Starost, Matthew F., Huang, Huang‐Chiao, Mueller, Jenna L.

“…While surgical resection is a mainstay of cancer treatment, many tumors are unresectable due to stage, location, or comorbidities. Ablative therapies, which…”
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Late multiple organ surge in interferon-regulated target genes characterizes staphylococcal enterotoxin B lethality by Ferreyra, Gabriela A, Elinoff, Jason M, Demirkale, Cumhur Y, Starost, Matthew F, Buckley, Marilyn, Munson, Peter J, Krakauer, Teresa, Danner, Robert L

“…Bacterial superantigens are virulence factors that cause toxic shock syndrome. Here, the genome-wide, temporal response of mice to lethal intranasal…”
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Mouse Prkar1a haploinsufficiency leads to an increase in tumors in the Trp53+/− or Rb1+/− backgrounds and chemically induced skin papillomas by dysregulation of the cell cycle and Wnt signaling by Almeida, Madson Q., Muchow, Michael, Boikos, Sosipatros, Bauer, Andrew J., Griffin, Kurt J., Tsang, Kit Man, Cheadle, Chris, Watkins, Tonya, Wen, Feng, Starost, Matthew F., Bossis, Ioannis, Nesterova, Maria, Stratakis, Constantine A.

“…PRKAR1A inactivation leads to dysregulated cAMP signaling and Carney complex (CNC) in humans, a syndrome associated with skin, endocrine and other tumors. The…”
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Ambient oxygen promotes tumorigenesis by Sung, Ho Joong, Ma, Wenzhe, Starost, Matthew F, Lago, Cory U, Lim, Philip K, Sack, Michael N, Kang, Ju-Gyeong, Wang, Ping-yuan, Hwang, Paul M

“…Oxygen serves as an essential factor for oxidative stress, and it has been shown to be a mutagen in bacteria. While it is well established that ambient oxygen…”
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Alternate protein kinase A activity identifies a unique population of stromal cells in adult bone by Tsang, Kit Man, Starost, Matthew F, Nesterova, Maria, Boikos, Sosipatros A, Watkins, Tonya, Almeida, Madson Q, Harran, Michelle, Li, Andrew, Collins, Michael T, Cheadle, Christopher, Mertz, Edward L, Leikin, Sergey, Kirschner, Lawrence S, Robey, Pamela, Stratakis, Constantine A

“…A population of stromal cells that retains osteogenic capacity in adult bone (adult bone stromal cells or aBSCs) exists and is under intense investigation…”
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Identification and Characterization of SSTK, a Serine/Threonine Protein Kinase Essential for Male Fertility by Spiridonov, Nikolay A., Wong, Lily, Zerfas, Patricia M., Starost, Matthew F., Pack, Svetlana D., Paweletz, Cloud P., Johnson, Gibbes R.

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Xanthine Oxidoreductase Is an Endogenous Regulator of Cyclooxygenase-2 by Ohtsubo, Toshio, Rovira, Ilsa I, Starost, Matthew F, Liu, Chengyu, Finkel, Toren

“…Xanthine oxidoreductase (XOR) is the enzyme responsible for the final step in purine degradation resulting in the generation of uric acid. Here we have…”
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Development of Neurological Disease Is Associated with Increased Immune Activation in Simian Immunodeficiency Virus-Infected Macaques by QUE DANG, WHITTED, Sonya, BUCKLER-WHITE, Alicia, HIRSCH, Vanessa M, GOEKEN, Robert M, BRENCHLEY, Jason M, MATSUDA, Kenta, BROWN, Charles R, LAFONT, Bernard A. P, STAROST, Matthew F, LYENGAR, Ranjini, PLISHKA, Ronald J

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CD4, but not Cxcr6, is necessary for control of Pneumocystis murina infection by Bishop, Lisa R., Starost, Matthew F., Kovacs, Joseph A.

“…CD4+ T cells are critical to control of Pneumocystis infection, and Cxcr6 has been shown to be upregulated in these cells during infection, but the roles of…”
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Normoglycemia alone is insufficient to prevent long-term complications of hepatocellular adenoma in glycogen storage disease type Ib mice by Yiu, Wai Han, Pan, Chi-Jiunn, Mead, Paul A, Starost, Matthew F, Mansfield, Brian C, Chou, Janice Y

“…Background/Aims Glycogen storage disease type Ib (GSD-Ib) patients deficient in a glucose-6-phosphate transporter (G6PT) manifest disturbed glucose…”
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