James Orengo
M.D./Ph.D. Student, Molecular and Cellular Biology
Funding: Predoctoral NRSA from NIH/NINDS
jo044504@bcm.tmc.edu
713-798-5021
Joined the lab: June 2005
Research Interests
Myotonic Dystrophy (DM) is the most common form of adult onset muscular dystrophy. Individuals afflicted with this disease have expanded tri-nucleotide repeats located within a non-coding fragment of the DMPK gene. Expression of these repeats leads to a toxic accumulation of CUG RNA repeats within the nucleus, which is hypothesized to be responsible for a trans-dominant misregulation of alternative splicing. A hallmark of this multi-systemic disorder is a debilitating, progressive wasting of skeletal muscle. Using an inducible tissue-specific mouse model, I am working on unraveling the pathogenic mechanisms leading to skeletal muscle atrophy in DM. I am also searching for novel proteins that interact with expanded CUG RNA molecules, which may provide further insight into why alternative splicing is misregulated in DM.
Publications
Orengo J.P., Chambon P., Metzger D., Mosier D.R., Snipes G.J., and Cooper T.A. “Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy.” Proc Natl Acad Sci 2008. 105(7):2646-51
Orengo, J.P. and Cooper, T.A. Alternative splicing in disease in Alternative splicing in the post-genomic era, B.R. Graveley and B. Blencowe, ed. 2007 Landes publishing.
Orengo, J.P., Bundman, D., and Cooper, T.A. “A bichromatic fluorescent reporter for cell-based screens of alternative splicing.” Nucleic Acids Research 2006 Vol. 34, No. 22