Pearce Lab

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Primary Research Focus

Research in the Pearce Lab focuses on understanding the molecular basis of several inherited pediatric neurodegenerative diseases, including the infantile, late infantile and juvenile onset forms of Batten disease.

Dr. Pearce and his team use mouse and miniature pig models of these rare, fatal diseases to reveal molecular and cellular pathomechanisms, to identify new therapeutic targets and to test new therapeutic approaches.

Primary Research Group

Pediatrics and Rare Diseases

Secondary Research Groups

(605) 312-6004

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About the Pearce Lab

About David Pearce, Scientist

Dr. David Pearce has over 20 years of experience in biomedical research. His active research program focuses on the pediatric neurodegenerative disorder Batten disease.

Dr. Pearce wrote more than 90 publications on Batten disease, used yeast and animal models to elucidate the basis of this disease, and developed a number of reagents that lend themselves to further study as a means to determine the pathophysiology of Batten disease.

BS: University of University of Wolverhampton, Wolverhampton, England (1983-1986)
PhD: University of Bath, Bath, England (1986-1989)
Postdoctoral Fellow: University of Rochester, Rochester, NY (1990-1993)
Postdoctoral Research Assistant: University of Oxford, Oxford, England (1993-1994)

Academic Affiliations and Training

Academic Appointments:

  • Professor, Department of Pediatrics, University of South Dakota Sanford School of Medicine (2009-present)

Administrative Roles:

  • Internal Advisory Committee, Developmental Research Program for Medical Students Grant, T35, South Dakota (2017-present)
  • Sanford Research Board of Directors (2015-present)
  • Co-Chair, Division of Research, Department of Pediatrics, University of South Dakota (2009-2015)
  • University of South Dakota and Mayo Clinic Departments of Pediatrics, T. Denny Sanford Grants Oversight Committee (2011-2015)

Highlighted Publications

 Johnson TB, Langin LM, Zhao J, Weimer JM, Pearce DA, Kovács AD. Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water. Sci Rep. 2019 Oct 18;9(1):14962. doi: 10.1038/s41598-019-51488-z. PubMed PMID: 31628420; PubMed Central PMCID: PMC6802212.

Johnson TB, Cain JT, White KA, Ramirez-Montealegre D, Pearce DA, Weimer JM. Therapeutic landscape for Batten disease: current treatments and future prospects. Nat Rev Neurol. 2019 Mar;15(3):161-178. doi: 10.1038/s41582-019-0138-8. Review. PubMed PMID: 30783219; PubMed Central PMCID: PMC6681450.

Beraldi R, Meyerholz DK, Savinov A, Kovács AD, Weimer JM, Dykstra JA, Geraets RD, Pearce DA. Genetic ataxia telangiectasia porcine model phenocopies the multisystemic features of the human disease. Biochim Biophys Acta Mol Basis Dis. 2017 Nov;1863(11):2862-2870. doi: 10.1016/j.bbadis.2017.07.020. Epub 2017 Jul 23. PubMed PMID: 28746835; PubMed Central PMCID: PMC5687068.

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Lab Projects and News

Mouse models of Batten disease

Mutations in the CLN1, CLN2 and CLN3 genes cause infantile, late infantile and juvenile Batten diseases, respectively. We use mouse models of these diseases that carry common disease-causing human mutations (Cln3ex7/8, Cln3Q352X, Cln1R151X, Cln2R207X), to test new therapeutic approaches (e.g., nonsense suppression therapy and drugs affecting glutamate neurotransmission).

Pig models of Batten disease

Since the mouse models of Batten disease have limitations, we generated Yucatan miniature pig models of juvenile (CLN3) and late infantile (CLN2) Batten diseases. The physiology of the pig is similar to humans, and therefore, the pig models human diseases more precisely than mice. Characterization of the CLN3 and CLN2 mutant pigs is in progress.

Meet the Pearce Lab Team

Attila Kovács, PhD

Assistant Research Scientist

Dr. Attila Kovács holds a PhD in cell biology from Eötvös Loránd University in Budapest, Hungary, and completed postdoctoral training at the Karolinska Institute in Stockholm, Sweden, and the University of Rochester in Rochester, New York. He has extensive research experience in both pharmaceutical and academic environments with expertise in neuroscience and behavioral pharmacology. Attila works on several projects, including: a) testing various pharmacotherapeutic approaches in mouse models of Batten disease; and b) investigating the role of the bacterial flora living in the gut (gut microbiota) in the pathophysiology of Batten disease using mouse models.

Vicki Swier, PhD

Staff Scientist

Dr. Vicki Swier holds a PhD from Texas Tech University, Lubbock, Texas, and completed postdoctoral training at Creighton University in Omaha, Nebraska. She has extensive research experience with miniature pig disease models. Vicki currently works on the characterization of pig models of CLN2 Batten disease and Neurofibromatosis type 1 (NF1).

Logan Langin, BA

Senior Research Specialist

Logan Langin earned a bachelor’s degree in biology from Augustana University in Sioux Falls, South Dakota. He oversees and manages all laboratory activities, orders, mouse colony maintenance and laboratory projects. Logan is involved in the analysis of behavioral, histopathological and molecular phenotypes in therapeutic studies in mouse models of Batten disease.