Publications

1. Bruns., C.K., et al., Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants. Embo J., 2007.26(3): p. 855-66.

2. DeLaBarre, B., Christianson, J.C., et al., Central pore residues mediate the p97/VCP activity required for ERAD. Molecular Cell, 2006. 22(4): p. 451-62.

3. Ryu, K.Y., et al., Ubiquitin-specific protease 2 as a tool for quantification of total ubiquitin levels in biological specimens. Anal Biochem., 2006. 353(1): p. 153-5.

4. Iwata, A., et al., HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin. Journal of Biological Chemistry, 2005. 280(48): p. 40282-92.

5. Iwata, A.., et al., Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation. Proc Natl Acad Sci USA., 2005. 102(37): p. 13135-40.

6. Bennett, E.J., et al., Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. Molecular Cell, 2005. 17(3): p. 351-65.

7. Gilchrist, C.A., et al., Effect of ubiquitin expression on neuropathogenesis in a mouse model of familial amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol., 2005. 31(1): p. 20-33.

8. Rajan, R.S., et al., Suppression of wild-type rhodopsin maturation by mutants linked to autosomal dominant retinitis pigmentosa. Journal of Biological Chemistry, 2005. 280(2): p. 1284-91.

9. Kopito, R.R., The missing linker: An unexpected role for a histone deacetylase. Molecular Cell, 2003. 12(6): p. 1349-1351.

10. Fayadat, L. and R.R. Kopito, Recognition of a single transmembrane degron by sequential quality control checkpoints. Molecular Biology of the Cell, 2003. 14(3): p. 1268-1278.

11. Elkabetz, Y., et al., Immunoglobulin light chains dictate vesicular transport-dependent and -independent routes for IgM degradation by the ubiquitin-proteasome pathway. Journal of Biological Chemistry, 2003. 278(21): p. 18922-18929.

12. Bence, N.F. and R.R. Kopito, Protein unfolding and the ubiquitin proteasome system: implications for neurodegenerative disease. Faseb Journal, 2003. 17(5): p. A792-A792.

13. Rajan, R.S., et al., Specificity in intracellular protein aggregation and inclusion body formation. Biophysical Journal, 2002. 82(1): p. 322a-322a.

14. Rajan, R.S., et al., A rhodopsin mutant linked to retinitis pigmentosa is aggregation prone, interacts with the ubiquitin proteasome pathway and induces misfolding the wild-type protein. Molecular Biology of the Cell, 2002. 13: p. 287a-287a.

15. Lenk, U., et al., A role for mammalian Ubc6 homologues in ER-associated protein degradation. Journal of Cell Science, 2002. 115(14): p. 3007-3014.

16. Johnston, J.A., M.E. Illing, and R.R. Kopito, Cytoplasmic dynein/dynactin mediates the assembly of aggresomes. Cell Motility and the Cytoskeleton, 2002. 53(1): p. 26-38.

17. Illing , M.E. , et al., A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system. Journal of Biological Chemistry, 2002. 277(37): p. 34150-34160.

18. Harrington, M.A. and R.R. Kopito, Cysteine residues in the nucleotide binding domains regulate the conductance state of CFTR channels. Biophysical Journal, 2002. 82(3): p. 1278-1292.

19. Gelman, M.S., E.S. Kannegaard, and R.R. Kopito, A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator. Journal of Biological Chemistry, 2002. 277(14): p. 11709-11714.

20. Gelman, M.S. and R.R. Kopito, Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis. Journal of Clinical Investigation, 2002. 110(11): p. 1591-1597.

21. Fayadat, L. and R.R. Kopito, Checkpoints for transmembrane protein folding in the secretory pathway. Journal of General Physiology, 2002. 120(1): p. 3a-3a.

22. Bence, N.F., R.M. Sampat, and R.R. Kopito, Impairment of the ubiquitin-proteasome system by protein aggregation. Science, 2001. 292(5521): p. 1552-1555.

23. Kopito, R.R. and D. Ron, Conformational disease. Nature Cell Biology, 2000. 2(11): p. E207-E209.

24. Kopito, R.R., Aggresomes, inclusion bodies and protein aggregation. Trends in Cell Biology, 2000. 10(12): p. 524-530.

25. Kopito, R.R. and R. Sitia, Aggresomes and Russell bodies - Symptoms of cellular indigestion? Embo Reports, 2000. 1(3): p. 225-231.

26. Johnston, J.A., et al., Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proceedings of the National Academy of Sciences of the United States of America , 2000. 97(23): p. 12571-12576.

27. Gilchrist , C.A. , et al., Characterization of ubiquitin-proteasome pathway involvement in the neurodegenerative process in a mouse model of amyotrophic lateral sclerosis. Molecular Biology of the Cell, 2000. 11: p. 402a-402a.

28. Bence, N.F., R. Sampat, and R.R. Kopito, The use of an in vivo proteasome reporter to study the effects of CFTR and Huntingtin inclusions on the ubiquitin proteasome pathway. Molecular Biology of the Cell, 2000. 11: p. 118a-118a.

29. Yu, H. and R.R. Kopito, The role of multiubiquitination in dislocation and degradation of the alpha subunit of the T cell antigen receptor. Journal of Biological Chemistry, 1999. 274(52): p. 36852-36858.

30. Johnston, J.A., C.L. Ward, and R.R. Kopito, Aggresomes: A cellular response to misfolded proteins. Faseb Journal, 1999. 13(7): p. A1520-A1520.

31. Harrington, M.A., K.L. Gunderson, and R.R. Kopito, Redox reagents and divalent cations alter the kinetics of cystic fibrosis transmembrane conductance regulator channel gating. Journal of Biological Chemistry, 1999. 274(39): p. 27536-27544.

32. Sato, S., C.L. Ward, and R.R. Kopito, Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro. Journal of Biological Chemistry, 1998. 273(13): p. 7189-7192.

33. Johnston, J.A., C.L. Ward, and R.R. Kopito, Aggresomes: A cellular response to misfolded proteins. Journal of Cell Biology, 1998. 143(7): p. 1883-1898.

34. Zeng, W.Z., et al., Immuno and functional characterization of CFTR in submandibular and pancreatic acinar and duct cells. American Journal of Physiology-Cell Physiology, 1997. 42(2): p. C442-C455.

35. Yu, H., et al., Cytosolic degradation of T-cell receptor alpha chains by the proteasome. Journal of Biological Chemistry, 1997. 272(33): p. 20800-20804.

36. Kopito, R.R., ER quality control: The cytoplasmic connection. Cell, 1997. 88(4): p. 427-430.

37. Kopito, R.R. and C.L. Ward, Hot papers - Protein degradation - Degradation of CFTR by the ubiquitin-proteasome pathway by C.L. Ward, S. Omura, R.R. Kopito - Comments. Scientist, 1997. 11(9): p. 12-12.