Mueller-Steiner Sarah, Zhou Yungui, Arai Hideaki, Roberson Erik D, Sun Binggui, Chen Jennifer, Wang Xin, Yu Guiqiu, Esposito Luke, Mucke Lennart, Gan Li
Gladstone Institute of Neurological Disease, University of California, San Francisco, 1650 Owens Street, 94158, USA.
Neuron. 2006 Sep 21;51(6):703-14. doi: 10.1016/j.neuron.2006.07.027.
Alzheimer's disease (AD) may result from the accumulation of amyloid-beta (Abeta) peptides in the brain. The cysteine protease cathepsin B (CatB) is associated with amyloid plaques in AD brains and has been suspected to increase Abeta production. Here, we demonstrate that CatB actually reduces levels of Abeta peptides, especially the aggregation-prone species Abeta1-42, through proteolytic cleavage. Genetic inactivation of CatB in mice with neuronal expression of familial AD-mutant human amyloid precursor protein (hAPP) increased the relative abundance of Abeta1-42, worsening plaque deposition and other AD-related pathologies. Lentivirus-mediated expression of CatB in aged hAPP mice reduced preexisting amyloid deposits, even thioflavin S-positive plaques. Under cell-free conditions, CatB effectively cleaved Abeta1-42, generating C-terminally truncated Abeta peptides that are less amyloidogenic. Thus, CatB likely fulfills antiamyloidogenic and neuroprotective functions. Insufficient CatB activity might promote AD; increasing CatB activity could counteract the neuropathology of this disease.
阿尔茨海默病(AD)可能源于大脑中β-淀粉样蛋白(Aβ)肽的积累。半胱氨酸蛋白酶组织蛋白酶B(CatB)与AD大脑中的淀粉样斑块有关,并且一直被怀疑会增加Aβ的产生。在此,我们证明CatB实际上通过蛋白水解切割降低了Aβ肽的水平,尤其是易于聚集的Aβ1-42。在具有家族性AD突变型人淀粉样前体蛋白(hAPP)神经元表达的小鼠中,CatB的基因失活增加了Aβ1-42的相对丰度,加剧了斑块沉积和其他与AD相关的病理变化。慢病毒介导的CatB在老年hAPP小鼠中的表达减少了已有的淀粉样沉积物,甚至是硫黄素S阳性斑块。在无细胞条件下,CatB有效地切割Aβ1-42,产生C末端截短的Aβ肽,这些肽的淀粉样生成能力较低。因此,CatB可能具有抗淀粉样生成和神经保护功能。CatB活性不足可能会促进AD的发生;增加CatB活性可能会对抗这种疾病的神经病理学。