Pearce Lab

Castaneda, J. and Pearce, D. A. (2008) Identification of α-fetoprotein as an autoantigen in juvenile Batten disease. Neurobiol. Dis. 29, 92-102

Kovács, A. D, and Pearce, D. A. (2008) Attenuation of AMPA receptor activity improves motor coordination in a mouse model of Juvenile Batten disease.
Exp. Neurol. 209, 288-91

Adams, H.R., J. Kwon, F.J. Marshall, E. A. de Blieck, D. A. Pearce and J. W. Mink. (2007) Neuropsychological symptoms of Juvenile-onset Batten Disease (CLN3): Experiences from two studies. Child. Neurol. 22, 621-7.

Ramirez-Montealegre, D., and Pearce, D. A. (2007) Imaging of late infantile neuronal ceroid lipofuscinosis: a clinical rating scale. Neurology 69(6):503-4.

Weimer, J. M, Benedict, J.W., Elshatory, E., Short, D.S., Ramirez-Montealegre, D., Ryan, D., Alexander, N., Federoff, H.J., Cooper, J.D., and Pearce, D. A.(2007) Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of Juvenile Neuronal Ceroid Lipofuscinosis. Brain. Res. 1162:98-112.

Benedict, JW, Sommers, C and Pearce DA (2007) Progressive Oxidative Damage in the CNS of a Murine Model for Juvenile Batten Disease. J. Neurosci. Res. 85(13):2882-91.

Osório, NS, Carvalho, A, Almeida, AJ, Padilla-Lopez, S, Leão, C, Laranjinha, J, Ludovico, P, Pearce, DA and Rodrigues, F. (2007) Nitric oxide signaling is disrupted in the yeast model for Batten disease. Mol. Biol. Cell. 18(7):2755-67.

Phillips-Vitiello, S.N., Wolfe, D.M. and Pearce, D. A. (2007). Absence of Btn1p in the yeast model for juvenile Batten disease may cause arginine to become toxic to yeast cells. Hum. Mol. Genet. 16(9):1007-16.

Lim, M. J., Alexander, N., Benedict, J.W., Chattopadhyay, S., Shemilt, S.J., Guerin, C.J., Cooper, J. D. and Pearce, D. A., (2007) IgG Entry and Deposition are Components of the Neuroimmune Response in Batten Disease. Neurobiol of Disease.25: 239-251.

Zimmerman, A.W., Connors, S.L., Metteson, K.J., Lee, L.C., Singer, H.S., Castaneda, J. and Pearce, D. A.. (2007) Maternal Antibrain Antibodies in Autism. Brain, Behav. and Immunol. 21: 351-357.

Lim, M. J., Beake, J., Bible, E., Curran, T. M., Ramirez-Montealegre, D., Pearce, D. A., and Cooper, J. D. (2006) Distinct Patterns of Serum Immunoreactivity as Evidence for Multiple Brain Directed Autoantibodies in Juvenile Ceroid Lipofuscinosis. Neuropath. Appl. Neurobiol. 32: 469-482.

Leman, A.R.., Polochock, S., Mole, S. E., Pearce D. A. and Rothberg, P. G. (2006) Homogeneous PCR Nucleobase Quenching Assays to Detect Four Mutations that Cause Neuronal Ceroid Lipofuscinosis: T75 and R151X in CLN1, and IVS5-1G>C and R208X in CLN. J. Neurosci. Meth. 157 (15): 124-131.

Ramirez-Montealegre, D., Rothberg P.G., and Pearce, D. A. (2006) Another disorder finds its gene. Brain. 129(6):1353-1356.

Padilla-Lopez, S. and Pearce D. A. (2006) Saccharomyces cerevisiae lacking Btn1p modulate vacuolar ATPase activity in order to regulate pH imbalance in the vacuole. J. Biol. Chem. Vol. 281: 10273-10280.

Kovács, A. D, Weimer, J. M, and Pearce D. A. (2006) Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease. Neurobiol. Dis. Vol. 22(3): 575-585.

Weimer, J. M, Custer, A. W, Benedict, J. W, Alexander, N. A, Kingsley, E, Federoff, H. J, Cooper , J. D, and Pearce, D. A. (2006) Visual Deficits in a Mouse Model of Batten Disease Are the Result of Optic Nerve Degeneration and Loss of Dorsal Lateral Geniculate Thalamic Neurons. Neurobiol. Dis. Vol. 22(2): 284-293.

Adams., H., deBlieck., E. A., Mink., J. W., Marshall., F. J., Kwon., J. M., Dure, L., Rothberg, P. G., Ramirez-Montealegre, D and Pearce, D. A. (2006) Standardized Assessment of Behavior and Adaptive Living Skills in Juvenile Neuronal Ceroid Lipofuscinosis. Develop. Med. and Child Neurol. 48(4): 259-64.

Pears MR, Cooper JD, Mitchison HM, Mortishire-Smith RJ, Pearce DA, Griffin JL.(2005) High resolution 1H NMR Based Metabolomics Indicates a Neurotransmitter Cycling Deficit in Cerebral Tissue from a Mouse Model of Batten Disease. J Biol Chem. 280: 42508-42514.

Ramirez-Montealegre D, Pearce DA. (2005) Defective Lysosomal Arginine Transport in Juvenile Batten Disease. Hum Mol Genet. 14: 3759-3773.

Kwon., J. M., Rothberg, P. G., Leman, A. R., Weimer, J. M., Mink., J. W and Pearce, D. A. (2005) Novel CLN3 Mutation Predicted to Cause Complete Loss of Protein Function Does Not Modify the Classical JNCL Phenotype. Neurosci. Lett. 387(2):111-4.

Marshall, F. J., De Blieck, E. A., Mink, J. W., Dure, L., Adams, H., Messing, S., Rothberg, P. G., Levy, E., McDonough, T., DeYoung, J., Wang, M., Ramirez-Montealegre, D., Kwon, J. M and Pearce, D. A. (2005) A clinical rating scale for Batten disease: Reliable and relevant for clinical trials. Neurology 65(2):275-9.

Kim, Y., Chattopadhyay, S., Locke, S. J and Pearce, D. A. (2005) Interaction among Btn1p, Btn2p and Ist2p reveals potential interplay between the vacuole, amino acid levels and ion homeostasis in the yeast Saccharomyces cerevisiae. Euk. Cell. 4, 281-288.

Leman, A. R., Pearce, D. A and Rothberg, P. G. (2005) Gene symbol: CLN3. Disease: juvenile neuronal ceroid lipofuscinosis (Batten disease). Hum Genet. 116, 236.

Weimer, J. M., Chattopadhyay, S., Custer, A and Pearce, D.A. (2005) Elevation of Hook1 in a disease model of Batten disease does not affect a novel interaction between Ankyrin G and Hook1. Biochem. Biophys. Res. Comm. 330, 1176-1181.

Cho, S. K., Gao, N., Pearce, D. A., Lehrman, M. A. and Hofmann, S. L. (2005) Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology. 15, 637-648.

Fatemi S. H., Snow, A. V., Stary, J. M., Araghi-Niknam, M., Reutiman, T. J., Lee, S., Brooks, A. I. and Pearce, D. A. (2005). Reelin signaling is impaired in autism. Biol Psychiatry. 57, 777-787.

Ramirez-Montealegre, D., Chattopadhyay, S., Curran, T. M., Wasserfall, C., Pritchard, L., Schatz, D., Petitto, J., Hopkins, D., She, J-X., Rothberg, P. G., Atkinson, M. and Pearce, D. A. (2005). Autoimmunity to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease. Neurology. 64, 743-745.

Rothberg, P. G., Ramirez-Montealegre, D., Frazier, S. D., Pearce, D. A. (2004) Homogeneous PCR Nucleobase Quenching Assay to Detect the 1-kbp Deletion in CLN3 That Causes Batten Disease. J. Mol. Diag. 6, 260-263.

Chattopadhyay, S., Kingsley, E., Serour, A., Curran, T. M., Brooks, A. I., and Pearce, D. A. (2004). Altered Gene Expression in the Eye of a Mouse Model for Batten Disease. IOVS. 45, 2893-2905.

Pontikis, C. C., Cella, C. V., Parihar, N., Lim, M. J., Chakrabarti, S., Mitchison, H. M., Mobley, W. C., Rezaie, P., Pearce, D. A and Cooper, J. D. (2004). Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation. Brain Research. 1023, 231-242.

Kim, Y., Ramirez-Montealegre, D. and Pearce D. A. (2003). A role in vacuolar arginine transport for yeast Btn1p, and human CLN3, the protein defective in Batten disease. Proc. Natl. Acad. Sci. 100, 15458-15462.

Chattopadhyay, S, Curran, T. M, McCall, K, Mooney, R. A. and Pearce, D. A. (2003). Altered Amino Acid Levels in Sera of a Mouse Model for Juvenile Neuronal Ceroid Lipofuscinoses. Clin. Chem. Acta. 332, 145-148.

Sappington, R. M., Pearce, D. A. and Calkins, D. C. (2003). Optic Nerve Degeneration in a Murine Model of Juvenile Ceroid Lipofuscinosis. IOVS. 44, 3725-3731.

Elshatory, Y, Brooks, A. I, Chattopadhyay, S, Curran, T. M, Gupta, P, Ramalingam, V, Hofmann, S. L. and Pearce, D. A. (2003). Early Changes in Gene Expression in Two Models of Batten Disease. FEBS Lett. 538, 207-212.

Brooks, A. I, Chattopadhyay, S, Mitchison, H. M, Nussbaum, R. L and Pearce, D.A. (2003). Functional Categorization of Gene Expression Changes in the Cerebellum of a Cln3-Knockout Mouse Model for Batten Disease. Mol. Gen Metab. 78, 17-30.

Chattopadhyay, S, Roberts, P. M. and Pearce D. A. (2003). The yeast model for Batten Disease: A role for Btn2p in the trafficking of the golgi-associated vesicular targeting protein, Yif1p. Biochem. Biophys. Res. Comm. 302, 534-538.

Chattopadhyay, S., Kricenski-Perry, E., Wenger, David A. and Pearce D. A. (2002). An autoantibody to GAD65 in sera of patients with juvenile neuronal ceroid lipofuscinoses. Neurology . 59, 1816-1817.

Bijlani, R., Chen, Y., Brooks, A. I., Pearce D. A. and Ogihara, M. (2002). Prediction of biologically significant components from microarray data: Independently Consistent Expression Discriminator (ICED). Bioinformatics. 19, 62-70.

Kricenski-Perry, E., Applegate, C. D., Serour, A., Mhyre, T. R., Leonardo, C. C. and Pearce, D. A. (2002). Altered flurothyl seizure induction latency, phenotype and subsequent mortality in a mouse model of juvenile neuronal ceroid lipofuscinoses/Batten disease. Epilepsia. 43, 1137-40.

Chattopadhyay, S., Ito, M., Cooper, J. D., Brooks, A. I., Curran, T. M., Powers, J. M. and Pearce, D. A. (2002). An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegeneartive disorder Batten disease. Hum. Mol. Genet. 11 , 1421-1431.

Chattopadhyay, S and Pearce D. A. (2002). Interaction with Btn2p is required for localization of Rsg1p: Btn2p-Mediated Changes in Arginine Uptake in Saccharomyces cerevisiae. Euk. Cell. 1 , 606-612.

Cottone CD, Chattopadhyay S, Pearce DA. Searching for interacting partners of CLN1, CLN2 and Btn1p with the two-hybrid system. Europ J Paediatr Neurol. 2001; 5 Suppl A:95-8.

Chattopadhyay S, Muzaffar NE, Sherman F, Pearce DA. The yeast model for batten disease: mutations in BTN1, BTN2, and HSP30 alter pH homeostasis. J Bacteriol. 2000 Nov;182(22):6418-23.

S. Chattopadhyay and D. A. Pearce(2000) Neural and Extraneural Expression of the Neuronal Ceroid Lipofuscinoses Genes CLN1, CLN2, and CLN3: Functional Implications for CLN3. Mol Genet Metab 71 : 207-211

R. E. Haskell, C. J. Carr, D. A. Pearce, M. J. Bennett and B. L. Davidson(2000) Batten disease: evaluation of CLN3 mutations on protein localization and function. Hum Mol Genet 9 : 735-44

D. A. Pearce, C. J. Carr, B. Das and F. Sherman(1999) Phenotypic reversal of the btn1 defects in yeast by chloroquine: a yeast model for Batten disease. Proc Natl Acad Sci U S A 96 : 11341-5

D. A. Pearce, T. Ferea, S. A. Nosel, B. Das and F. Sherman(1999) Action of BTN1, the yeast orthologue of the gene mutated in Batten disease. Nat Genet 22 : 55-8

D. A. Pearce, S. A. Nosel and F. Sherman(1999) Studies of pH regulation by Btn1p, the yeast homolog of human Cln3p. Mol Genet Metab 66 : 320-3

D. A. Pearce and F. Sherman(1999) Investigation of Batten disease with the yeast Saccharomyces cerevisiae. Mol Genet Metab 66 : 314-9

D. A. Pearce and F. Sherman(1998) A yeast model for the study of Batten disease. Proc Natl Acad Sci U S A 95 : 6915-8

D. A. Pearce and F. Sherman (1997).  BTN1, a Yeast Gene Corresponding to the Human Gene Responsible for Batten's Disease, is not Essential for Viability, Mitochondrial Function, or Degradation of Mitochondrial ATP-Synthase.  Yeast. 13 , 691-697.