title: Large animal models for Batten disease: a review.
abstract: The neuronal ceroid lipofuscinoses, collectively referred to as Batten disease, make up a group of inherited childhood disorders that result in blindness, motor and cognitive regression, brain atrophy, and seizures, ultimately leading to premature death. So far more than 10 genes have been implicated in different forms of the neuronal ceroid lipofuscinoses. Most related research has involved mouse models, but several naturally occurring large animal models have recently been discovered. In this review, we discuss the different large animal models and their significance in Batten disease research.
authors.affiliation: Sanford Children's Health Research Center, Sanford Research/University of South Dakota, Sioux Falls, SD, USA.
authors.forename: Krystal
authors.initials: K
authors.lastname: Weber
authors.forename: David A
authors.initials: DA
authors.lastname: Pearce
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology*; D002417:Cattle; D004195:Disease Models, Animal; D004285:Dogs; D008565:Membrane Proteins / Q000235:genetics*; D009154:Mutation; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics* / Q000473:pathology; D012756:Sheep
chemical_list: D008565:Membrane Proteins
keywords: Batten disease; ONCL; animal models; neuronal ceroid lipofuscinosis