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.
About the Pearce Lab
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 (Cln3ex7/8, 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
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.