Thomas L. Shirley

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Msh2 deficiency prevents in vivo somatic instability of the CAG repeat in Huntington disease transgenic mice

Kevin Manley, Thomas L. Shirley, Lorraine Flaherty, Anne Messer
Nature Genetics
1999

Huntington disease (HD), an autosomal dominant, progressive neurodegenerative disorder, is caused by an expanded CAG repeat sequence leading to an increase in the number of glutamine residues in the… (More)

Interannual abundance patterns of copepods during an ENSO event in Icy Strait, southeastern Alaska

Wongyu Park, Molly V. Sturdevant, +4 authors Thomas L. Shirley
2004

Interannual copepod abundance (number m ) and sea surface temperatures (SST, 2-m depth) were examined from four stations in Icy Strait, southeastern Alaska, monthly from May to September, 1997e2002.… (More)

A human neuronal tissue culture model for Lesch-Nyhan disease.

Thomas L. Shirley, J Chris Lewers, +5 authors Hamida Amirali Jinnah
Journal of neurochemistry
2007

Mutations in the gene encoding the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HPRT) cause Lesch-Nyhan disease, a neurodevelopmental disorder characterized by cognitive,… (More)

Hypoxanthine-guanine phosphoribosyl transferase regulates early developmental programming of dopamine neurons: implications for Lesch-Nyhan disease pathogenesis.

Irène Ceballos-Picot, Lionel Mockel, +5 authors Hamida Amirali Jinnah
Human molecular genetics
2009

Hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency results in Lesch-Nyhan disease (LND), where affected individuals exhibit a characteristic neurobehavioral disorder that has been… (More)

A Hybrid Life-Cycle Assessment of Nonrenewable Energy and Greenhouse-Gas Emissions of a Village-Level Biomass Gasification Project in China

Changbo Wang, Lixiao Zhang, Shuying Yang, Mingyue Pang, Thomas L. Shirley
2012

Small-scale bio-energy projects have been launched in rural areas of China and are considered as alternatives to fossil-fuel energy. However, energetic and environmental evaluation of these projects… (More)

Building a fisheries trophic interaction dataBase for management and modeling research in the gulf of mexico large marine ecosystem

James D. Simons, May Yuan, +11 authors Jorrit H. Poelen
2013

development of ecosystem-based fisheries management models depends, to a large extent, on the availability of trophic interaction data. These models could address ecological questions, examine… (More)

Epigenetic memory and parliamentary privilege combine to evoke discussions on inheritance

Understanding the basis of epigenetic memory is a fast-moving challenge in modern biology. At a recent Company of Biologists Workshop held at Steyning’s historic Wiston House, thirty researchers led… (More)

Altered membrane NTPase activity in Lesch-Nyhan disease fibroblasts: comparison with HPRT knockout mice and HPRT-deficient cell lines.

Cibele S. Pinto, Hyder Azad Jinnah, Thomas L. Shirley, William L. Nyhan, Roland Seifert
Journal of neurochemistry
2005

Lesch-Nyhan disease (LND) is a rare disorder caused by a defect of an enzyme in the purine salvage pathway, hypoxanthine phosphoribosyl transferase (HPRT). It is still unknown how the metabolic… (More)

Paroxysmal dyskinesias in mice.

Thomas L. Shirley, Lekha M Rao, Ellen J Hess, Hamida Amirali Jinnah
Movement disorders : official journal of the…
2008

Animal models of human disease are important tools for revealing the underlying mechanisms of pathophysiology and developing therapeutic strategies. Several unique mouse calcium channel mutants have… (More)

DNA vaccination against mutant huntingtin ameliorates the HDR6/2 diabetic phenotype.

Todd W Miller, Thomas L. Shirley, William J. Wolfgang, Xiaowei Kang, Anne Messer
Molecular therapy : the journal of the American…
2003

Immunization against extracellular neurotoxic proteins has shown promise in the treatment of several neurodegenerative disorders. We sought to determine whether immunization against mutant… (More)

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