文献类型： 外文期刊
作者： Yang, Qiang 1 ; Zhao, Xueyan 1 ; Xing, Yuyun 1 ; Jiang, Chao 1 ; Jiang, Kai 1 ; Xu, Pan 1 ; Liu, Weiwei 1 ; Ren, Jun 1 ; Hua 1 ;
作者机构： 1.Jiangxi Agr Univ, Coll Anim Sci & Technol, State Key Lab Pig Genet Improvement & Prod Techno, Nanchang 330045, Jiangxi, Peoples R China
2.Shandong Acad Agr Sci, Inst Anim Sci & Vet Med, Shandong Prov Key Lab Anim Dis Control & Breeding, Jinan 250100, Shandong, Peoples R China
关键词： MPS IIIB; Lysosomal storage disease; NAGLU; Pig model
期刊名称：BIOLOGY OPEN （ 影响因子：2.422； 五年影响因子：2.647 ）
ISSN： 2046-6390
年卷期： 2018 年 7 卷 10 期
页码：
收录情况： SCI
摘要： Mucopolysaccharidosis type IIIB (MPS IIIB) is a rare genetic disorder caused by loss-of-function mutations in the NAGLU gene. Pigs are an ideal large animal model for human diseases; however, a porcine model of MPS IIIB has not been reported. We have previously generated a heterozygous NAGLU-deficient (NAGLU(+/-)) Large White boar via a transgenic approach. Here we characterized phenotypes of the F-1 offspring of this founder to establish a pig model for MPS IIIB. qRT-PCR revealed that the NAGLU expression level was significantly decreased in a variety of tissues in NAGLU(+/-) pigs. ELISA assays showed obvious deficiency of NAGLU and higher (P<0.05) glycosaminoglycan levels in multiple tissues from NAGLU(+/-)pigs. NAGLU(+/-) pigs grew at a significantly (P<0.05) slower rate than control animals (NAGLUl. Death, mostly sudden death, occurred at all ages in NAGLU(+/-) pigs, most of which died within two years. Necropsy findings included pleural adhesions, lung shrinkage and abnormalities in the pericardium and mild hepatomegaly in NAGLU(+/-) pigs. Notable pathological changes were observed in the sections of brain, liver, spleen and kidney from NAGLU(+/-) pigs. Brain atrophy, ventriculomegaly, cerebellar atrophy and abnormalities in the intracerebral capsule, parietal lobes and the thalamus were also evident in NAGLU(+/-) pigs. Together, NAGLU(+/-) pigs show typical symptoms of human MPS IIIB patients and thus represent a novel large animal model for the disease.
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