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103. Expression and characterization of the cystic fibrosis transmembrane conductance regulator

Author

Gregory, Richard J., Cheng, Seng H., Rich, Devra P., Marshall, John, Supreme Court justice, Paul, Sucharita, Hehir, Kathleen, Ostedgaard, Lynda, Klinger, Katherine W., Welsh, Michael J., and Smith, Alan E.

Subjects
Proteins -- Analysis, Cystic fibrosis -- Physiological aspects, Recombinant DNA -- Research, Environmental issues, Science and technology, Zoology and wildlife conservation
Abstract

Cystic fibrosis (CF) is the most common deadly inheritable disease among Caucasians. CF is characterized by defective salt transport in organs, particularly the lungs, sweat glands, intestines, and pancreas. The major cellular defect in CF patients involves the function and regulation of chloride channels. These channels, which are proteins embedded in the membranes of epithelial cells (which form the exterior layers of most body surfaces), allow the movement of chloride ions from the cell interior to the exterior. In normal epithelial cells, the chloride channel opens when it is phosphorylated (modified by the addition of phosphate) by a kinase (a type of enzyme). In CF cells, chloride channels are present but they cannot be activated by the kinase, and thus cannot secrete chloride ions or fluid. A major research finding in 1989 was the isolation of a gene which had a mutation in 70 percent of patients with CF. The gene codes for a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), but the actual function of the protein has not yet been determined. This article describes work done with the gene to allow synthesis of the protein from the gene information by means of laboratory techniques. The results show that the protein is a membrane-associated protein to which sugar molecules attach during synthesis, which is common for membrane-bound proteins. In addition, this synthesized, or recombinant, CFTR can be phosphorylated by the kinase, which regulates chloride channels (as well as other proteins). Antibodies that recognize the native protein also recognize recombinant CFTR. These results indicate that recombinant CFTR is structurally and functionally equivalent to CFTR, and this will allow CFTR function to be studied with the aim of improving diagnosis and therapy of cystic fibrosis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1990

104. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

Author

Rich, Devra P., Anderson, Matthew P., Gregory, Richard J., Cheng, Seng H., Paul, Sucharita, Jefferson, Douglas M., McCann, John D., Klinger, Katherine W., Smith, Alan E., and Welsh, Michael J.

Subjects
Cystic fibrosis -- Physiological aspects, Chloride channels -- Physiological aspects, Cystic fibrosis -- Genetic aspects, Environmental issues, Science and technology, Zoology and wildlife conservation
Abstract

Cystic fibrosis (CF) is the most common deadly inheritable disease affecting Caucasians. CF is characterized by defective salt transport in organs, particularly the lungs, sweat glands, intestines, and pancreas. The major cellular defect in CF patients involves the function and regulation of chloride channels. These channels, which are proteins embedded in the membranes of epithelial cells (which form the exterior layers of most body surfaces), allow the movement of chloride ions from the cell interior to the exterior. In normal epithelial cells, the chloride channel opens when it is phosphorylated (modified by the addition of phosphate) by a kinase (a type of enzyme). In CF cells, chloride channels are present but they cannot be activated by the kinase, and thus cannot secrete chloride ions or fluid. A major research finding in 1989 was the isolation of a gene which contained a mutation in 70 percent of patients with CF. The gene codes for a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and work is ongoing to determine the actual function of this protein. The results described in this article show that when CFTR is added to CF cells, which have the mutated CFTR and are thereby defective in chloride transport, the defect in chloride secretion is corrected. This demonstrates a causal relationship between CFTR mutations and the actual dysfunctions associated with CF. The results also suggest that CFTR is either a chloride channel or that it regulates chloride channels, but it cannot be determined which of these two possibilities is true. The study suggests that treatment of CF by correcting the underlying defect may some day be feasible. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1990

105. Substrate Reduction Therapy for Sandhoff Disease through Inhibition of Glucosylceramide Synthase Activity

Author

Marshall, John, primary, Nietupski, Jennifer B., additional, Park, Hyejung, additional, Cao, James, additional, Bangari, Dinesh S., additional, Silvescu, Cristina, additional, Wilper, Terry, additional, Randall, Kristen, additional, Tietz, Drew, additional, Wang, Bing, additional, Ying, Xiaoyou, additional, Leonard, John P., additional, and Cheng, Seng H., additional

Published
2019
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110. Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo

Author

Hu Jim, Chow Yu-Hua, Hyde Stephen C, Gill Deborah R, Smith Rbecca L, Painter Hazel, Somerton Luci, Singh Charanjit, Farley Raymond, Garcia Sara, Kitson Chris, Griesenbach Uta, Gray Mike, Edbrooke Mark, Ogilvie Varrie, MacGregor Gordon, Scheule Ronald K, Cheng Seng H, Caplen Natasha J, and Alton Eric WFW

Subjects
Diseases of the respiratory system, RC705-779
Abstract

Abstract Background The cationic lipid Genzyme lipid (GL) 67 is the current "gold-standard" for in vivo lung gene transfer. Here, we assessed, if GL67 mediated uptake of siRNAs and asODNs into airway epithelium in vivo. Methods Anti-lacZ and ENaC (epithelial sodium channel) siRNA and asODN were complexed to GL67 and administered to the mouse airway epithelium in vivo Transfection efficiency and efficacy were assessed using real-time RT-PCR as well as through protein expression and functional studies. In parallel in vitro experiments were carried out to select the most efficient oligonucleotides. Results In vitro, GL67 efficiently complexed asODNs and siRNAs, and both were stable in exhaled breath condensate. Importantly, during in vitro selection of functional siRNA and asODN we noted that asODNs accumulated rapidly in the nuclei of transfected cells, whereas siRNAs remained in the cytoplasm, a pattern consistent with their presumed site of action. Following in vivo lung transfection siRNAs were only visible in alveolar macrophages, whereas asODN also transfected alveolar epithelial cells, but no significant uptake into conducting airway epithelial cells was seen. SiRNAs and asODNs targeted to β-galactosidase reduced βgal mRNA levels in the airway epithelium of K18-lacZ mice by 30% and 60%, respectively. However, this was insufficient to reduce protein expression. In an attempt to increase transfection efficiency of the airway epithelium, we increased contact time of siRNA and asODN using the in vivo mouse nose model. Although highly variable and inefficient, transfection of airway epithelium with asODN, but not siRNA, was now seen. As asODNs more effectively transfected nasal airway epithelial cells, we assessed the effect of asODN against ENaC, a potential therapeutic target in cystic fibrosis; no decrease in ENaC mRNA levels or function was detected. Conclusion This study suggests that although siRNAs and asODNs can be developed to inhibit gene expression in culture systems and certain organs in vivo, barriers to nucleic acid transfer in airway epithelial cells seen with large DNA molecules may also affect the efficiency of in vivo uptake of small nucleic acid molecules.

Published
2006
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119. A randomised, double-blind, placebo-controlled trial of repeated nebulisation of non-viral cystic fibrosis transmembrane conductance regulator (CFTR) gene therapy in patients with cystic fibrosis

Author

Alton, Eric Wfw, Armstrong, David K, Ashby, Deborah, Bayfield, Katie J, Bilton, Diana, Bloomfield, Emily V, Boyd, A Christopher, Brand, June, Buchan, Ruaridh, Calcedo, Roberto, Carvelli, Paula, Chan, Mario, Cheng, Seng H, Collie, David S, Cunningham, Steve, Davidson, Heather E, Davies, Gwyneth, Davies, Jane C, Davies, Lee A, Dewar, Maria H, Doherty, Ann, Donovan, Jackie, Dwyer, Natalie S, Elgmati, Hala I, Featherstone, Rosanna F, Gavino, Jemyr, Gea-sorli, Sabrina, Geddes, Duncan M, Gibson, James Sr, Gill, Deborah R, Greening, Andrew P, Griesenbach, Uta, Hansell, David M, Harman, Katharine, Higgins, Tracy E, Hodges, Samantha L, Hyde, Stephen C, Hyndman, Laura, Innes, J Alastair, Jacob, Joseph, Jones, Nancy, Keogh, Brian F, Limberis, Maria P, Lloyd-evans, Paul, Maclean, Alan W, Manvell, Michelle C, Mccormick, Dominique, Mcgovern, Michael, Mclachlan, Gerry, Meng, Cuixiang, Montero, M Angeles, Milligan, Hazel, Moyce, Laura J, Murray, Gordon D, Nicholson, Andrew G, Osadolor, Tina, Parra-leiton, Javier, Porteous, David J, Pringle, Ian A, Punch, Emma K, Pytel, Kamila M, Quittner, Alexandra L, Rivellini, Gina, Saunders, Clare J, Scheule, Ronald K, Sheard, Sarah, Simmonds, Nicholas J, Smith, Keith, Smith, Stephen N, Soussi, Najwa, Soussi, Samia, Spearing, Emma J, Stevenson, Barbara J, Sumner-jones, Stephanie G, Turkkila, Minna, Ureta, Rosa P, Waller, Michael D, Wasowicz, Marguerite Y, Wilson, James M, and Wolstenholme-hogg, Paul

Published
2016

120. Environmentally Benign Material Removal Processes for the Fabrication of Microdevices

Author

Steven Danyluk, Travis Lee Blackburn, Leo Cheng Seng, and David Lee Butler

Subjects
Heat-affected zone, Fabrication, Materials science, Mechanical Engineering, Polishing, Nanotechnology, Condensed Matter Physics, Surface micromachining, Electrokinetic phenomena, Machining, Mechanics of Materials, Etching, General Materials Science, Nanometre
Abstract

Non-contact material removal processes offer numerous advantages over traditional machining approaches and nowhere is this more apparent than in the fabrication of micro devices. Current micromachining techniques such as microgrinding and micromilling have limitations with respect to their positioning accuracy and tool deflections. Electro thermal processes such as microEDM and laser machining usually result in a heat affected zone being produced. Other approaches such as etching and non-contact ultraprecision polishing are either costly or are not suitable for high throughput. In order to address these limitations, alternative micromachining techniques are required. In this paper, a non-contact material removal technique based on the electrokinetic phenomenon is proposed for precise material removal at rates in the order of nanometers/min. The aim of this research is to have a better understanding on the electrokinetic material removal technique by studying the trajectory of the particles and the influence of the frequency of the electric field on the material removal rate.

Published
2009

122. Characteristics of Minimally Oversized Adeno-Associated Virus Vectors Encoding Human Factor VIII Generated Using Producer Cell Lines and Triple Transfection

Author

Nambiar, Bindu, primary, Cornell Sookdeo, Cathleen, additional, Berthelette, Patricia, additional, Jackson, Robert, additional, Piraino, Susan, additional, Burnham, Brenda, additional, Nass, Shelley, additional, Souza, David, additional, O'Riordan, Catherine R., additional, Vincent, Karen A., additional, Cheng, Seng H., additional, Armentano, Donna, additional, and Kyostio-Moore, Sirkka, additional

Published
2017
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126. Fibrillin-1 Regulates Skeletal Stem Cell Differentiation by Modulating TGFβ Activity Within the Marrow Niche

Author

Smaldone, Silvia, Clayton, Nicholas P, del Solar, Maria, Pascual, Gemma, Cheng, Seng H, Wentworth, Bruce M, Schaffler, Mitchell B, and Ramirez, Francesco

Subjects
Mice, Knockout, Mice, Bone Marrow, Transforming Growth Factor beta, Fibrillin-1, Microfilament Proteins, Animals, Cell Differentiation, Mesenchymal Stem Cells, Stem Cell Niche, Fibrillins, Article
Abstract

A full understanding of the microenvironmental factors that control the activities of skeletal stem cells (also known as mesenchymal stem cells [MSCs]) in the adult bone marrow holds great promise for developing new therapeutic strategies to mitigate age-related diseases of bone and cartilage degeneration. Bone loss is an understudied manifestation of Marfan syndrome, a multisystem disease associated with mutations in the extracellular matrix protein and TGFβ modulator fibrillin-1. Here we demonstrate that progressive loss of cancellous bone in mice with limbs deficient for fibrillin-1 (Fbn1(Prx1-/-) mice) is accounted for by premature depletion of MSCs and osteoprogenitor cells combined with constitutively enhanced bone resorption. Longitudinal analyses of Fbn1(Prx1-/-) mice showed incremental bone loss and trabecular microarchitecture degeneration accompanied by a progressive decrease in the number and clonogenic potential of MSCs. Significant paucity of marrow fat cells in the long bones of Fbn1(Prx1-/-) mice, together with reduced adipogenic potential of marrow stromal cell cultures, indicated an additional defect in MSC differentiation. This postulate was corroborated by showing that an Fbn1-silenced osteoprogenitor cell line cultured in the presence of insulin yielded fewer than normal adipocytes and exhibited relatively lower PPARγ levels. Consonant with fibrillin-1 modulation of TGFβ bioavailability, cultures of marrow stromal cells from Fbn1(Prx1-/-) limb bones showed improper overactivation of latent TGFβ. In line with this finding, systemic TGFβ neutralization improved bone mass and trabecular microarchitecture along with normalizing the number of MSCs, osteoprogenitor cells, and marrow adipocytes. Collectively, our findings show that fibrillin-1 regulates MSC activity by modulating TGFβ bioavailability within the microenvironment of marrow niches.

Published
2015

127. Effect of Baseline or Changes in Adrenergic Activity on Clinical Outcomes in the β-Blocker Evaluation of Survival Trial

Author

L. Olson, Michael R. Bristow, Phillip W. Lavori, Cheng Seng Liang, Robert Zelis, Steven K. Krueger, Brack G. Hattler, Brian D. Lowes, R. Nuzzo, Heidi Krause-Steinrauf, Michael J. Domanski, Surai Thaneemit-Chen, William T. Abraham, E. J. Eichhorn, H. S. Loeb, Joshua M. Hare, and JoAnn Lindenfeld

Subjects
Male, Sympathetic Nervous System, Adrenergic beta-Antagonists, Adrenergic, law.invention, Propanolamines, Norepinephrine (medication), Norepinephrine, chemistry.chemical_compound, Randomized controlled trial, Predictive Value of Tests, Antiadrenergic agent, law, Sympatholytic, Physiology (medical), Humans, Medicine, Survival analysis, Aged, Heart Failure, Likelihood Functions, business.industry, Bucindolol, Stroke Volume, Middle Aged, medicine.disease, Survival Analysis, Hospitalization, Treatment Outcome, chemistry, Anesthesia, Heart failure, Female, Cardiology and Cardiovascular Medicine, business, Biomarkers, medicine.drug
Abstract

Background— Adrenergic activation is thought to be an important determinant of outcome in subjects with chronic heart failure (CHF), but baseline or serial changes in adrenergic activity have not been previously investigated in a large patient sample treated with a powerful antiadrenergic agent. Methods and Results— Systemic venous norepinephrine was measured at baseline, 3 months, and 12 months in the β-Blocker Evaluation of Survival Trial (BEST), which compared placebo treatment with the β-blocker/sympatholytic agent bucindolol. Baseline norepinephrine level was associated with a progressive increase in rates of death or death plus CHF hospitalization that was independent of treatment group. On multivariate analysis, baseline norepinephrine was also a highly significant ( P Conclusions— In BEST, a subset of patients treated with bucindolol had an increased risk of death as the result of sympatholysis, which compromised the efficacy of this third-generation β-blocker.

Published
2004

129. CNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease

Author

Marshall, John, primary, Sun, Ying, additional, Bangari, Dinesh S, additional, Budman, Eva, additional, Park, Hyejung, additional, Nietupski, Jennifer B, additional, Allaire, Amy, additional, Cromwell, Mary A, additional, Wang, Bing, additional, Grabowski, Gregory A, additional, Leonard, John P, additional, and Cheng, Seng H, additional

Published
2016
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130. 621. Widespread Gene Delivery to the Nonhuman Primate Brain for the Treatment of Huntington's Disease

Author

Hadaczek, Piotr, primary, Stanek, Lisa, additional, Ciesielska, Agnieszka, additional, Pivirotto, Philip, additional, O'Riordan, Catherine, additional, Mastis, Bryan, additional, Sebastian, Waldy San, additional, Forsayeth, John, additional, Cheng, Seng H., additional, Bankiewicz, Krystof S., additional, and Shihabuddin, Lamya S., additional

Published
2016
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131. 92. Evaluation of Producer Cell Line Platform for Production of Oversized AAV-FVIII Vectors

Author

Nambiar, Bindu, primary, Sookdeo, Cathleen, additional, Berthelette, Patricia, additional, Jackson, Robert, additional, Burnham, Brenda, additional, Nass, Shelley, additional, Souza, David, additional, O'Riordan, Catherine R., additional, Vincent, Karen, additional, Cheng, Seng H., additional, Armentano, Donna, additional, and Kyostio-Moore, Sirkka, additional

Published
2016
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132. 301. AAV Capsid Engineering to Improve Transduction in Retina and Brain

Author

Sullivan, Jennifer, primary, Stanek, Lisa, additional, Lukason, Michael, additional, Barry, Elizabeth, additional, Russell, Shayla, additional, Morris, James, additional, Mastis, Bryan, additional, Alves, Anna, additional, Bu, Jie, additional, Shihabuddin, Lamya S., additional, Cheng, Seng, additional, and Scaria, Abraham, additional

Published
2016
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133. 249. Preliminary Results of a Phase 1, Open-Label, Safety and Tolerability Study of a Single Intravitreal Injection of AAV2-sFLT01 in Patients with Neovascular Age-Related Macular Degeneration

Author

Scaria, Abraham, primary, LeHalpere, Annaig, additional, Purvis, Annie, additional, Delacono, Cheryl, additional, Cheng, Seng, additional, Wadsworth, Samuel, additional, Campochiaro, Peter, additional, Heier, Jeff, additional, and Buggage, Ronald, additional

Published
2016
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135. Glucocerebrosidase modulates cognitive and motor activities in murine models of Parkinson’s disease

Author

Rockenstein, Edward, primary, Clarke, Jennifer, additional, Viel, Catherine, additional, Panarello, Nicholas, additional, Treleaven, Christopher M., additional, Kim, Changyoun, additional, Spencer, Brian, additional, Adame, Anthony, additional, Park, Hyejung, additional, Dodge, James C., additional, Cheng, Seng H., additional, Shihabuddin, Lamya S., additional, Masliah, E., additional, and Sardi, S. Pablo, additional

Published
2016
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138. Glucosylceramide synthase inhibition reduces α-synuclein pathology and improves cognition in murine models of synucleinopathy

Author

Pablo Sardi, S., primary, Viel, Catherine, additional, Clarke, Jennifer, additional, Treleaven, Christopher, additional, Park, Hyejung, additional, Dodge, James, additional, Marshall, John, additional, Cromwell, Mandy, additional, Leonard, John, additional, Wang, Bing, additional, Cheng, Seng H., additional, and Shihabuddin, Lamya, additional

Published
2016
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140. Widespread AAV1- and AAV2-mediated transgene expression in the nonhuman primate brain: implications for Huntington's disease

Author

Hadaczek, Piotr, primary, Stanek, Lisa, additional, Ciesielska, Agnieszka, additional, Sudhakar, Vivek, additional, Samaranch, Lluis, additional, Pivirotto, Philip, additional, Bringas, John, additional, O'Riordan, Catherine, additional, Mastis, Bryan, additional, San Sebastian, Waldy, additional, Forsayeth, John, additional, Cheng, Seng H, additional, Bankiewicz, Krystof S, additional, and Shihabuddin, Lamya S, additional

Published
2016
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144. Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial

Author

Alton, Eric W F W, primary, Armstrong, David K, additional, Ashby, Deborah, additional, Bayfield, Katie J, additional, Bilton, Diana, additional, Bloomfield, Emily V, additional, Boyd, A Christopher, additional, Brand, June, additional, Buchan, Ruaridh, additional, Calcedo, Roberto, additional, Carvelli, Paula, additional, Chan, Mario, additional, Cheng, Seng H, additional, Collie, D David S, additional, Cunningham, Steve, additional, Davidson, Heather E, additional, Davies, Gwyneth, additional, Davies, Jane C, additional, Davies, Lee A, additional, Dewar, Maria H, additional, Doherty, Ann, additional, Donovan, Jackie, additional, Dwyer, Natalie S, additional, Elgmati, Hala I, additional, Featherstone, Rosanna F, additional, Gavino, Jemyr, additional, Gea-Sorli, Sabrina, additional, Geddes, Duncan M, additional, Gibson, James S R, additional, Gill, Deborah R, additional, Greening, Andrew P, additional, Griesenbach, Uta, additional, Hansell, David M, additional, Harman, Katharine, additional, Higgins, Tracy E, additional, Hodges, Samantha L, additional, Hyde, Stephen C, additional, Hyndman, Laura, additional, Innes, J Alastair, additional, Jacob, Joseph, additional, Jones, Nancy, additional, Keogh, Brian F, additional, Limberis, Maria P, additional, Lloyd-Evans, Paul, additional, Maclean, Alan W, additional, Manvell, Michelle C, additional, McCormick, Dominique, additional, McGovern, Michael, additional, McLachlan, Gerry, additional, Meng, Cuixiang, additional, Montero, M Angeles, additional, Milligan, Hazel, additional, Moyce, Laura J, additional, Murray, Gordon D, additional, Nicholson, Andrew G, additional, Osadolor, Tina, additional, Parra-Leiton, Javier, additional, Porteous, David J, additional, Pringle, Ian A, additional, Punch, Emma K, additional, Pytel, Kamila M, additional, Quittner, Alexandra L, additional, Rivellini, Gina, additional, Saunders, Clare J, additional, Scheule, Ronald K, additional, Sheard, Sarah, additional, Simmonds, Nicholas J, additional, Smith, Keith, additional, Smith, Stephen N, additional, Soussi, Najwa, additional, Soussi, Samia, additional, Spearing, Emma J, additional, Stevenson, Barbara J, additional, Sumner-Jones, Stephanie G, additional, Turkkila, Minna, additional, Ureta, Rosa P, additional, Waller, Michael D, additional, Wasowicz, Marguerite Y, additional, Wilson, James M, additional, and Wolstenholme-Hogg, Paul, additional

Published
2015
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145. Priapism in a Fabry disease mouse model is associated with upregulated penile nNOS and eNOS expression.

Author

Meng, Xing-Li, Arning, Erland, Wight-Carter, Mary, Day, Taniqua S., Jabbarzadeh-Tabrizi, Siamak, Chen, Shuyuan, Ziegler, Robin J., Bottiglieri, Teodoro, Schneider, Jay W., Cheng, Seng H., Schiffmann, Raphael, and Shen, Jin-Song

Abstract

Fabry disease is a glycosphingolipidosis caused by deficient activity of α-galactosidase A; it is one of a few diseases that are associated with priapism, an abnormal prolonged erection of the penis. The goal of this study was to investigate the pathogenesis of Fabry disease-associated priapism in a mouse model of the disease. We found that Fabry mice develop late-onset priapism. Neuronal nitric oxide synthase (nNOS), which was predominantly present as the 120-kDa N-terminus-truncated form, was significantly upregulated in the penis of 18-month-old Fabry mice compared to wild type controls (~fivefold). Endothelial NOS (eNOS) was also upregulated (~twofold). NO level in penile tissues of Fabry mice was significantly higher than wild type controls at 18 months. Gene transfer-mediated enzyme replacement therapy reversed abnormal nNOS expression in the Fabry mouse penis. The penile nNOS level was restored by antiandrogen treatment, suggesting that hyperactive androgen receptor signaling in Fabry mice may contribute to nNOS upregulation. However, the phosphodiesterase-5A expression level and the adenosine content in the penis, which are known to play roles in the development of priapism in other etiologies, were unchanged in Fabry mice. In conclusion, these data suggested that increased nNOS (and probably eNOS) content and the consequential elevated NO production and high arterial blood flow in the penis may be the underlying mechanism of priapism in Fabry mice. Furthermore, in combination with previous findings, this study suggested that regulation of NOS expression is susceptible to α- galactosidase A deficiency, and this may represent a general pathogenic mechanism of Fabry vasculopathy. [ABSTRACT FROM AUTHOR]

Published
2018
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149. Dimorphic Effects of Transforming Growth Factor-β Signaling During Aortic Aneurysm Progression in Mice Suggest a Combinatorial Therapy for Marfan Syndrome

Author

Cook, Jason R., primary, Clayton, Nicholas P., additional, Carta, Luca, additional, Galatioto, Josephine, additional, Chiu, Emily, additional, Smaldone, Silvia, additional, Nelson, Carol A., additional, Cheng, Seng H., additional, Wentworth, Bruce M., additional, and Ramirez, Francesco, additional

Published
2015
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150. Antisense oligonucleotide-mediated suppression of muscle glycogen synthase 1 synthesis as an approach for substrate reduction therapy of Pompe disease

Author

Clayton, Nicholas P., primary, Nelson, Carol A., additional, Weeden, Timothy, additional, Taylor, Kristin M., additional, Moreland, Rodney J., additional, Scheule, Ronald K., additional, Leger, Andrew J., additional, Phillips, Lucy, additional, Cheng, Seng H., additional, and Wentworth, Bruce M., additional

Published
2015
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