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Original Articles

Meyer, M., Kovacs, A.D., Pearce, D.A. Decreased sensitivity of palmitoyl protein thioesterase 1-deficient neurons to chemical anoxia. Metab Brain Dis. 2017 Feb;32(1):275-279.

Fietz, M., AlSayed M., Burke, D., Cohen-Pfieffer, J, Cooper, J.D., Dvorakova, L, Giugliani, R., Izzo, E., Jahnova, H., Lukacs, Z., Mole, S.E., Noher de Halac, I., Pearce, D.A., Poupetova, H., Schulz, A., Specchio, N., Xin, W., Miller, N. Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis. Mol Genet Metab. 2016 Sep;119(1-2):160-7.

Hersrud, S.L., Kovacs. A.D., Pearce, D.A. (2016) Antigen presenting cell abnormalities in the Cln3-/-mouse model of juvenile neuronal ceroid lipofuscinosis. Biochim Biophys Acta. 2016 Jul;1862(7):1324-36.

Simpkins, J.A., Rickel, K.E., Madeo, M., Ahlers, B.A., Carlisle, G.B., Nelson, H.J., Cardillo, A.L., Weber, E.A, Vitiello, P.F., Pearce, D.A., Vitiello, S.P. (2016) Disruption of a cysteine transporter downregulates expression of genes involved in sulfer regulation and cellular respiration. Biol Open. 2016 Jun 15;5(6):689-97.

Thada V., Miller J. N., Kovacs, A. D. and Pearce D. A. (2016). Tissue specific variation of nonsense mutant transcript level and drug-induced read-through efficiency in the Cln1R151X mouse model for infantile neuronal ceroid lipofuscinoses. J Cell Mol Med. 20(2), 381-5.

Amolins, M.W., Ezrailson, C.N., Pearce, D.A., Elliott, A.J., Vitiello, P.F. (2015) Evaluating the effectiveness of a laboratory-based professional development program for science educators. Adv Physiol Educ. 39(4), 341-51.

Beraldi, R., Chan, C-H., Rogers, C. S., Kovacs, A. D., Meyerholz, D. K., Trantzas, C., Lambertz, A. M., Darbo, B. W., Weber, K. L., Whiye, K. A. M., Rheeden, R. V., Kruer, M. C., Dacken, B. A., Wang, X-J., Davis, B. T., Rohret, J. A., Struzynski, J. T., Rohret, F. A., Weimer, J. M and Pearce D. A. (2015). A novel porcine model of Ataxia Telangiectasia reproduces neurological features and motor deficits of human disease. Hum Mol Genet. 24(22), 6473-84.

Hersrud, S. L., Weber, K. L., Geraets, R. D., Chan, C-H. Pearce D. A. (2015) Plasma Biomarkers for Neuronal Ceroid Lipofuscinosis. FEBS J. 283(3), 459-71.

Kovács, A. D., Hof, C. and Pearce D. A. (2015) Abnormally increased surface expression of AMPA receptors in the cerebellum, cortex and striatum of Cln3-/- mice. Neurosci Lett. 607, 29-34.

Kovács, A. D. and Pearce D. A. (2015) Finding the most appropriate mouse model of juvenile CLN3 disease for therapeutic studies: the genetic background and gender really matter. Dis. Mod. Mech. 8, 351-361.

Madeo, M., Kovács, A. D. and Pearce D. (2014) The human synaptic vesicle protein, SV2A functions as a galactose transporter in Saccharomyces cerevisiae. J. Biol. Chem. 289, 33066-33071.

Miller, J., N, Kovacs, A. D. and Pearce D. A. (2014) The novel Cln1R151X mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy. Hum. Mol. Genet. 24, 185-196.

Dolisca SB, Mehta M, Pearce DA, Mink JW, Maria BL. (2013) Batten disease: clinical aspects, molecular mechanisms, translational science, and future directions. J Child Neurol. 28(9): 1074-100.

Finn R, Kovács AD, Pearce DA. (2013)  Treatment of the Ppt1-/- Mouse Model of Infantile Neuronal Ceroid Lipofuscinosis With the N-methyl-D-aspartate (NMDA) Receptor Antagonist Memantine. J Child Neurol. 28(9):1159-68.

Weber K, Pearce DA. (2013) Large animal models for batten disease: a review. J Child Neurol. 28(9):1123-7.

Kruer MC, Pearce DA, Orchard PJ, Steiner RD. (2013) Prospects for stem cell therapy in neuronal ceroid lipofuscinosis. Regen Med. 8(5):527-9. No abstract available

Drack AV, Miller JN, Pearce DA. (2013) A Novel c.1135_1138delCTGT Mutation in CLN3 Leads to Juvenile Neuronal Ceroid Lipofuscinosis.  J Child Neurol. 28(9):1112-6.

Miller JN, Pearce DA. (2013) A Novel c.776_777insA Mutation in CLN1 Leads to Infantile Neuronal Ceroid Lipofuscinosis.  J Child Neurol. 28(9):1106-11.

de Blieck EA, Augustine EF, Marshall FJ, Adams H, Cialone J, Dure L, Kwon JM, Newhouse N, Rose K, Rothberg PG, Vierhile A, Mink JW; Batten Study Group [Pearce DA]. (2013)  Methodology of clinical research in rare diseases: development of a research program in juvenile neuronal ceroid lipofuscinosis (JNCL) via creation of a patient registry and collaboration with patient advocates. Contemp Clin Trials. 35(2):48-54.

Getty A, Kovács AD, Lengyel-Nelson T, Cardillo A, Hof C, Chan CH, Pearce DA. (2013) Osmotic Stress Changes the Expression and Subcellular Localization of the Batten Disease Protein CLN3.  PLoS One. 8(6):e66203.

Dolisca SB, Mehta M, Pearce DA, Mink JW, Maria BL. (2013) Batten Disease: Clinical Aspects, Molecular Mechanisms, Translational Science, and Future Directions.  J Child Neurol. 28(9):1074-100.

Kriscenski-Perry E, Kovács AD, Pearce DA. (2013) Seizure Susceptibility, Phenotype, and Resultant Growth Delay in the nclf and mnd Mouse Models of Neuronal Ceroid Lipofuscinoses. J Child Neurol. 28(9):1137-41.

Kovács AD, Pearce DA. (2013) Location- and sex-specific differences in weight and motor coordination in two commonly used mouse strains.  Sci Rep. 3:2116.

Miller JN, Chan CH, Pearce DA. (2013) The role of nonsense-mediated decay in neuronal ceroid lipofuscinosis. Hum Mol Genet. 22(13):2723-34.

Kohan R, Carabelos M. N, Xin W, Sims K, Guelbert N, Cismondi I. A, Pons P, Alonso G. I, Troncoso M, Witting S, Pearce D. A, de Kremer R. D, Oller-Ramírez A. M, de Halac I. N (2013) 
Neuronal ceroid lipofuscinosis type CLN2: A new rationale for the construction of phenotypic subgroups based on a survey of 25 cases in South America.Gene. 516, 114-121.

Kovács, A. D., Saje, A., Wong, A., Ramji, S., Cooper, J. D. and Pearce D. A. (2012) Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease. Neuropharmacol. 63, 769-775

Finn, R., Kovács, A. D. and Pearce D. A. (2012). Altered glutamate receptor function in the cerebellum of the Ppt1-/- mouse, a murine model of infantile neuronal ceroid lipofuscinosis. J. Neurosci Res. 90, 367-375.

Padilla-López, S, Langager, D, Chan, C-H and Pearce D. A. (2012). Evidence for Btn1p involvement in phospholipid distribution in Saccharomyces cerevisiae. Dis. Mod. Mech. 5, 191-199.

Salek RM, Pears MR, Cooper JD, Mitchison HM, Pearce DA, Mortishire-Smith RJ, Griffin JL. (2011) A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses. Journal of Biomolecular NMR 49, 175-184.

Finn, R.,Kovács, A. D. and Pearce D. A. (2011) Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3?ex7/8-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis. Neurochem. Int. 58 (6): 648-655.

Kovács, A. D., Saje, A., Wong, A., Szenasi, G., Kiricisi, P., Szabo., Cooper, J. D., Pearce, D. A. (2010). Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease. Neuropharmacol. 60(2-3):405-9.

Pearce DA, Padilla-Lopez S (2011). Comment on Deletion of btn1, an orthologue of CLN3, increases glycolysis and perturbs amino acid metabolism in the fission yeast model of Batten disease. Mol Biosyst. 7(4):1347-8.

Seehafer, S. S., Ramirez-Montealegre., D, Wong, A ., Chan, C-H., Castaneda, J., Horak, M., Ahmadi, S. M., Lim, M. J., Cooper, J. D, and Pearce, D. A. (2011). Immunosuppression alters disease severity in juvenile Batten disease mice. J. NeuroImm. 230(1-2):169-72.

Getty, A. L., Benedict, J. W. and Pearce D. A. (2011). A Novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3-/- cells. Exp. Cell. Res.317(1):51-69

Wolfe, D. M., Padilla-Lopez, S., Phillips-Vitiello, S and Pearce D. A. (2011) pH-Dependent Localization of Btn1p in the Yeast Model for Batten Disease. Dis. Mod. Mech. 4(1):120-5

Finn, R., Kovács, A and Pearce D. A. (2010) Altered sensitivity to excitotoxic cell death and glutamate receptor expression between two commonly studied mouse strains. J. Neurosci Res. 88(12):2648-60

Phillips Vitiello, S. N., Benedict, J. W., Padilla-Lopez, S., Pearce, D. A. (2010) Interaction between Sdo1p and Btn1p in the S. cerevesiae model for Batten disease. Hum. Mol. Genet. 19(5):931-42.

Adams, H. R., Levy, E., Jordan, R., Kwon J.M., Marshall F. J., Vierhile, A., de Blieck E. A., Pearce D. A., Mink, J. W. (2010)  Genotype does not predict severity of behavioral phenotype in Juvenile Neuronal Ceroid Lipofuscinosis (Batten Disease). Develop. Med & Child Neurol.  Dev. Med. Child Neurol. 52(7):637-43.
 

Kielar, C., Wishart, T. M., Palmer, A., Dihanich, S., Wong, A. M., Macauley, S. L., Chan, C. H., Sands, M. S., Pearce, D. A., Cooper, J. D., Gillingwater, T. H. (2009) Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease. Hum Mol Genet. 18(21):4066-80. 

Seehafer, S. S. and Pearce, D. A. (2009). Spectral properties and mechanisms that underlie autofluorescent accumulations in Batten disease.
Biochem. Biophys. Res. Comm. 382(2):247-51

Weimer, J. M., Benedict, J. W., Getty, A. L., Pontikis, C. C., Lim, M. J.,  Cooper, J. D., and Pearce, D. A. (2009). Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.  Brain. Res. 126: 93-107.

Benedict, J. W., Getty, A. L. , Wishart, T.M., Gillingwater, T. H. and Pearce D. A. (2009) The protein product of the CLN6 gene responsible for variant late-onset infantile neuronal ceroid lipofuscinosis interacts with CRMP-2.   J. Neurosci. Res.87(9): 2157 - 2166.

Schmidt , K. A., Wolfe, D. M., Stiller, B. and Pearce, D. A. (2009). Cd 2+ , Mn 2+ , Ni 2+ and Se 2+ toxicity to Saccharomyces cerevisiae lacking YPK9p the orthologue of human ATP13A2. Biochem and Biophys Res Com. 383(2):198-202.

Osório, N. S., Sampaio-Marques, B., Chan, C-H., Oliveira, P., Pearce D. A., Sousa, N., and Rodrigues, F. (2009) Neurodevelopmental delay in the Cln3ex7/8 mouse model for Batten disease. Genes, Brain and Behavior. 8(3):337-45.

Chan, C-H. Ramirez-Montealegre, D and Pearce D. A. (2008) Altered Arginine Metabolism in the CNS of the Cln3-/- Mouse Model of Juvenile Batten Disease.  Neuropath and Appl. Neurobiol. 35(2):189-207.

Chan, C-H, Mitchison, H. M., and Pearce D. A. (2008).  Transcript and in silico analysis of CLN3 in Juvenile Neuronal Ceroid Lipofuscinoses and associated mouse models. Hum. Mol. Genet. 17(21): 3332-3339.

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, J. W. , Sommers, C. and Pearce D. A. (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.