Current Research Interests
We can learn a great deal about the basis of common neurodegenerative diseases by studying mutations that cause less common familial forms of disease.  One such familial disease is inclusion body myopathy with frontotemporal dementia and Paget’s disease of bone (IBMPFD). This multisystem neurodegenerative disease is caused by mutations in the gene for valosin-containing protein (VCP). We seek to further our understanding of this disease and the processes that govern neurodegeneration in the central nervous system and the periphery by using mouse, fly and cell culture models to tease apart the function of normal and mutant VCP.

Personal Background
I grew up in Bellingham, WA surrounded by the Cascade Mountains and the San Juan islands.  I bravely ventured “out East” for undergrad at Williams College, then made my way back to Seattle to spend the next few years pursuing a PhD at the University of Washington.  In addition to completing my degree, Seattle managed to provide a wonderful husband and was witness to the birth of our daughter Haley.  Our little family is happy to be in Philadelphia, where my husband is a busy Wharton student.

• Custer SK, Garden GA, Gill N, Rueb U, Libby RT, Schultz C, Guyenet SJ, Deller T, Westrum LE, Sopher BL, La Spada AR. Nat Neurosci. 2006 Oct;9(10):1302-11

• Taylor J, Grote SK, Xia J, Vandelft M, Graczyk J, Ellerby LM, La Spada AR, Truant R. Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal.
  J Biol Chem. 2006 Feb 3;281(5):2730-9.

• Chen S, Peng GH, Wang X, Smith AC, Grote SK, Sopher BL, La Spada AR. Interference of Crx-dependent transcription by ataxin-7 involves interaction between the glutamine regions and requires the ataxin-7 carboxy-terminal region for nuclear localization. Hum Mol Genet. 2004 Jan 1;13(1):53-67.

• Grote SK, La Spada AR. Insights into the molecular basis of polyglutamine neurodegeneration from studies of a spinocerebellar ataxia type 7 mouse model.
  Cytogenet Genome Res. 2003;100(1-4):164-74. Review.

• Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, La Spada AR. Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice. J Neurosci. 2002 Jun 15;22(12):4897-905.

• Schwartz PJ, Grote SK, Stephans KL, Adler EM. Zinc elevates neuropeptide Y levels in rat pheochromocytoma cells by a mechanism independent of L-channel mediated inhibition of release. Brain Res. 2000 Sep 15;877(1):12-22.