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Joshua A Benson

Explore the profile of Joshua A Benson including associated specialties, affiliations and a list of published articles. Areas
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Articles 5
Citations 73
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Recent Articles
1.
OReilly L, Benson J, Cummings E, Perlmutter D, Silverman G, Pak S
Expert Opin Drug Discov . 2014 Jul; 9(9):1021-32. PMID: 24998976
Introduction: Many human diseases result from a failure of a single protein to achieve the correct folding and tertiary conformation. These so-called 'conformational diseases' involve diverse proteins and distinctive cellular...
2.
Benson J, Cummings E, OReilly L, Lee M, Pak S
Methods . 2014 Jul; 68(3):529-35. PMID: 24990146
Recent breakthrough discoveries have shown that committed cell fates can be reprogrammed by genetic, chemical and environmental manipulations. The germline of the nematode Caenorhabditis elegans provides a tractable system for...
3.
Long O, Benson J, Kwak J, Luke C, Gosai S, OReilly L, et al.
Hum Mol Genet . 2014 May; 23(19):5109-22. PMID: 24838286
The accumulation of serpin oligomers and polymers within the endoplasmic reticulum (ER) causes cellular injury in patients with the classical form α1-antitrypsin deficiency (ATD). To better understand the cellular and...
4.
OReilly L, Long O, Cobanoglu M, Benson J, Luke C, Miedel M, et al.
Hum Mol Genet . 2014 May; 23(19):5123-32. PMID: 24838285
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 therapy currently available, manipulation of the proteostasis...
5.
Li J, Pak S, OReilly L, Benson J, Wang Y, Hidvegi T, et al.
PLoS One . 2014 Feb; 9(1):e87260. PMID: 24498058
The classical form of α1-antitrypsin deficiency (ATD) is associated with hepatic fibrosis and hepatocellular carcinoma. It is caused by the proteotoxic effect of a mutant secretory protein that aberrantly accumulates...