Review Articles


Geraets RD, Koh SY, Hastings ML, Kielian T, Pearce D.A., Weimer JM. Moving towards effective therapeutic strategies for Neuronal Ceroid Lipofuscinosis. Orphanet J Rare Dis. 2016 Apr 16;11(1):40.

Miller, J. N and Pearce D. A. (2014) Nonsense-Mediated Decay in Genetic Disease: Friend or Foe? Hum. Mutat, Rev. 762C, 52-64.

Surendran, K., Vitiello, S. P. and Pearce D. A. (2013) Lysosome dysfunction in the pathogenesis of kidney diseases. Pediatric. Nephrol. 29, 2253-2261. PMCID: PMC4018427

Weber, K and Pearce D. A. (2013) Large animal models for Batten disease: A review. J. Child. Neurol. 28, 1123-1127. PMCID: PMC4009683

Getty, A. L. and Pearce D. A. (2010). Interactions of the Proteins of Neuronal Ceroid Lipofuscinosis: Clues to Function. Cell. Mol. Life. Sci. In Press.

Bozorg S, Ramirez-Montealegre D, Chung M and Pearce DA. (2009) Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) and the Eye. Survey. Ophthalmol. 2009 Jul-Aug;54(4):463-71.

Muzaffar, N. E and Pearce D. A. (2008) Analysis of NCL Proteins from an Evolutionary Standpoint. Curr. Genom. 9, 115-136.

Castaneda, J., Lim, M.J., Cooper, J.D., and Pearce, D. A.. (2007) Immune System Irregularities In Lysosomal Storage Disorders. Acta Neuropathologica.Acta Neuropathol. 2008 115(2):159-74.

Getty, A.L., Rothberg, P.G., and Pearce, D. A. (2007) Diagnosis of Neuronal Ceroid Lipofuscinosis: Mutation Detection Strategies. Expert Opinion on Medical Diagnostics. Expert Opinion on Medical Diagnostics, 2007. Vol. 1, No. 3, Pages 351-362.

Phillips, S.N., Muzaffar, N., Codlin, S., Korey, C.A., Taschner, P.E.M., de Voer, G., Mole, S.E., and Pearce, D.A. (2006) Characterizing Pathogenic Processes in Batten Disease: Use of Small Eukaryotic Model Systems. Biochim Biophys Acta. 2006 1762(10):906-19.

Wolfe, D. M. and Pearce, D. A. (2006) Channeling Studies in Yeast: Yeast as a Model for Channelopathies?  Neurmolecular Medicine 8(3):279-306.

Seehafer, S. S. and Pearce, D. A. (2006) You Say Lipofuscin, We Say Ceroid: Defining Autofluorescent Storage Material. Neurobiol. Aging. 27(4):576-88.

Chan, C-H and and Pearce, D. A. (2005) What have microarrays told us about the neuronal ceroid lipofuscinoses? Curr. Genom. 6, 257-268.

Phillips, S. N., Benedict, J. W., Weimer J. M. and Pearce, D. A (2005). CLN3, the Protein Associated with Batten Disease: Structure, Function and Localization. J. Neursci. Res. 79, 573-583.

Pearce, D. A., Atkinson, M. and Tagle, D. A. (2004). Glutamic Acid Decarboxylase Autoimmunity in Batten Disease and Other Disorders. Neurology 63, 2001-2005.

Weimer, J. M., Kriscenski-Perry, E., Elshatory, E and Pearce, D. A. (2002). The Neuronal Ceroid Lipofuscinoses: Mutations in different proteins result in similar disease. NeuroMolecular Medicine. 1 , 111-124.

D. A. Pearce(2000) Localization and processing of CLN3, the protein associated to Batten disease: where is it and what does it do? J Neurosci Res 59 : 19-23

D. A. Pearce(1999) Hereditary spastic paraplegia: mitochondrial metalloproteases of yeast. Hum Genet 104 : 443-8