title: Functional disruption of the prion protein gene in cloned goats.
abstract: The cellular prion protein (PrPC), a membrane glycoprotein anchored to the outer surface of neurons, lymphocytes and other cells, is associated directly with the pathogenesis of the transmissible spongiform encephalopathies (TSEs) occurring mainly in humans, cattle, sheep and goats. Although mice lacking PrPC develop and reproduce normally and are resistant to scrapie infection, large animals lacking PrPC, especially those species in which TSE occurs naturally, are currently not available. Here, five live PRNP+/- goats cloned by gene targeting are reported. Detailed RNA-transcription and protein-expression analysis of one PRNP+/- goat showed that one allele of the caprine PRNP gene had been disrupted functionally. No gross abnormal development or behaviour could be seen in these PRNP+/- goats up to at least 3 months of age. These heterozygous PRNP+/- goats are ready to be used in producing homozygous PRNP-/- goats in which no PrPC should be expressed.
authors.affiliation: Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.|Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Graduate School of Chinese Academy of Sciences, 320 Yue-Yang Road, Shanghai, 200031, China.
authors.forename: Guohua
authors.initials: G
authors.lastname: Yu
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Jianquan
authors.initials: J
authors.lastname: Chen
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Huiqing
authors.initials: H
authors.lastname: Yu
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Siguo
authors.initials: S
authors.lastname: Liu
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Juan
authors.initials: J
authors.lastname: Chen
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Xujun
authors.initials: X
authors.lastname: Xu
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Hongying
authors.initials: H
authors.lastname: Sha
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Xufeng
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Guoxiang
authors.initials: G
authors.lastname: Wu
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Shaofu
authors.initials: S
authors.lastname: Xu
authors.affiliation: Shanghai Genon Bio-Engineering Co. Ltd, 88 Cai-Lun Road, Shanghai 201203, China.|Shanghai Transgenic Research Center, 88 Cai-Lun Road, Shanghai 201203, China.
authors.forename: Guoxiang
authors.initials: G
authors.lastname: Cheng
mesh_terms: D000682:Amyloid / Q000235:genetics; D000818:Animals; D030801:Animals, Genetically Modified; D002478:Cells, Cultured; D005347:Fibroblasts; D006041:Goats / Q000235:genetics*; D018621:PrPC Proteins / Q000235:genetics* / Q000378:metabolism; D011328:Prions; D011498:Protein Precursors / Q000235:genetics
chemical_list: D000682:Amyloid; D018621:PrPC Proteins; D011328:Prions; D011498:Protein Precursors