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E. Remmelink A. Aartsma‐Rus A. B. Smit M. Verhage M. Loos M. van Putten 《Genes, Brain & Behavior》2016,15(6):558-567
Duchenne muscular dystrophy (DMD) is a progressive muscle‐wasting disorder, caused by mutations in the DMD gene and the resulting lack of dystrophin. The DMD gene has seven promoters, giving rise to multiple full‐length and shorter isoforms. Besides the expression of dystrophin in muscles, the majority of dystrophin isoforms is expressed in brain and dystrophinopathy can lead to cognitive deficits, including intellectual impairments and deficits in executive function. In contrast to the muscle pathology, the impact of the lack of dystrophin on the brain is not very well studied. Here, we study the behavioral consequences of a lack of full‐length dystrophin isoforms in mdx mice, particularly with regard to domains of executive functions and anxiety. We observed a deficit in cognitive flexibility in mdx mice in the absence of motor dysfunction or general learning impairments using two independent behavioral tests. In addition, increased anxiety was observed, but its expression depended on the context. Overall, these results suggest that the absence of full‐length dystrophin in mice has specific behavioral effects that compare well to deficits observed in DMD patients. 相似文献
33.
林火是大兴安岭地区森林生态系统的重要影响因子,研究火灾对植物多样性和优势种多度长期影响,有助于火灾区域森林生态系统重建与管理。本研究以大兴安岭不同火烧年限(1~5、5~10、10~20、20~30、30~40和40~50年)48对配对样地(火烧样地与邻近未火烧对照样地)为研究对象,利用二者差值变化来探讨森林恢复年限对植物多样性指数影响,通过对乔灌草相对多度变化确认火灾恢复对优势种的影响。研究结果表明:(1)火烧与对照间乔木多样性和丰富度差值先降后升趋势,在10年左右最低,而恢复30~40年后与对照样地相当或更高。灌木与乔木变化趋势相似,但是变化趋势多达到统计学显著(P<0.05),灌木Shannon-wiener多样性指数和丰富度差值随年限增加而线性上升。草本Simpson多样性指数随火烧年限增加而直线下降,但是均匀度与丰富度没有出现线性变化。(2)乔灌草优势种变化趋势为:乔木层白桦(Betula platyphylla)在火烧5~30年占比均超过30%,在30年后占比不超过15%,同时兴安落叶松(Larix gmelinii)在30~40年占比超过50%;灌木层在0~30年均是越桔(Vaccinium vitis-idaea)占比最大,30年之后变为榛子(Corylus heterophylla),草本层5~30年均是小叶章(Deyeuxia angustifolia)占比最大,30年之后变为其他物种。对照样地乔木层主要是兴安落叶松,占比超过50%,灌木层主要是越桔(Vaccinium vitis-idaea),草本层主要是小叶章(Deyeuxia angustifolia)。整体来看,乔木火后恢复需要更长的时间,而灌木和草本火后恢复更快。植物多样性及优势种变化是研究其对生态服务功能(如碳汇)影响的基础,我们研究结果为天保工程后续实施及科学管理大兴安岭森林生态系统提供数据支撑。 相似文献
34.
Anil Chekuri Katarzyna Zientara‐Rytter Angel Soto‐Hermida Shyamanga Borooah Marina Voronchikhina Pooja Biswas Virender Kumar David Goodsell Caroline Hayward Peter Shaw Chloe Stanton Donita Garland Suresh Subramani Radha Ayyagari 《Aging cell》2019,18(6)
Late‐onset retinal degeneration (L‐ORD) is an autosomal dominant macular degeneration characterized by the formation of sub‐retinal pigment epithelium (RPE) deposits and neuroretinal atrophy. L‐ORD results from mutations in the C1q‐tumor necrosis factor‐5 protein (CTRP5), encoded by the CTRP5/C1QTNF5 gene. To understand the mechanism underlying L‐ORD pathology, we used a human cDNA library yeast two‐hybrid screen to identify interacting partners of CTRP5. Additionally, we analyzed the Bruch's membrane/choroid (BM‐Ch) from wild‐type (Wt), heterozygous S163R Ctrp5 mutation knock‐in (Ctrp5S163R/wt), and homozygous knock‐in (Ctrp5S163R/S163R) mice using mass spectrometry. Both approaches showed an association between CTRP5 and HTRA1 via its C‐terminal PDZ‐binding motif, stimulation of the HTRA1 protease activity by CTRP5, and CTRP5 serving as an HTRA1 substrate. The S163R‐CTRP5 protein also binds to HTRA1 but is resistant to HTRA1‐mediated cleavage. Immunohistochemistry and proteomic analysis showed significant accumulation of CTRP5 and HTRA1 in BM‐Ch of Ctrp5S163R/S163R and Ctrp5S163R/wt mice compared with Wt. Additional extracellular matrix (ECM) components that are HTRA1 substrates also accumulated in these mice. These results implicate HTRA1 and its interaction with CTRP5 in L‐ORD pathology. 相似文献
35.
Precipitation and seasonality affect grazing impacts on herbage nutritive values in alpine meadows on the Qinghai-Tibet Plateau 总被引:1,自引:0,他引:1
Aims
Grasslands used for animal husbandry are chosen depending on the nutritive values of dominant herbage species. However, the influence of grazing in combination with precipitation and growing season on the nutritive values of dominant species has not been explicated. 相似文献
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37.
Haoli Yu Junyan Li Xiaolong Hu Jiahao Feng Hao Wang Fei Xiong 《Journal of biochemical and molecular toxicology》2019,33(8)
Cynaroside is a flavonoid compound proved to possess antioxidant activity, but its protective effect on age‐related macular degeneration still remains unclear. In this study, the protective effects of cynaroside on oxidative stress and apoptosis in retinal pigment epithelial (RPE) cells induced by hydrogen peroxide (H2O2) were investigated. Results showed that cynaroside effectively attenuated the decrease of cell activity induced by H2O2. The total reactive oxygen species can be remitted by decreasing malondialdehyde level, as well as increasing glutathione level, and superoxide dismutase and catalase activities. In addition, Western blot analysis indicated that cynaroside protected ARPE‐19 cells from apoptosis through downregulation of caspase‐3 protein activation which was controlled by the upstream proteins Bcl‐2 and Bax. It was finally proved that cynaroside could enhance the antioxidant and antiapoptotic ability in ARPE‐19 cells by promoting the expression of p‐Akt. 相似文献
38.
海州湾鱼类群落平均营养级和大型鱼类指数的变化特征 总被引:1,自引:0,他引:1
为探究近年来海州湾鱼类群落的变化特征,本研究根据2011年及2013-2017年春季(5月)和秋季(9-10月)在海州湾及其邻近海域进行的渔业资源底拖网调查,分析了海州湾鱼类群落平均营养级(MTL)和大型鱼类指数(LFI),对海州湾鱼类群落结构特征进行研究.结果表明:海州湾的优势鱼种主要有大泷六线鱼、方氏云鳚、尖海龙、小黄鱼、长蛇鲻等,且优势鱼种季节性变化明显.海州湾鱼类群落的平均营养级存在明显的年际和季节变化,总体上秋季的MTL高于春季,而且秋季MTL变化具有滞后性.LFI计算结果表明,近年来海州湾及其邻近海域大个体鱼类资源量有所减少,鱼类群落结构呈现出明显的小型化趋势. 相似文献
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40.
Dystroglycan regulates structure, proliferation and differentiation of neuroepithelial cells in the developing vertebrate CNS 总被引:1,自引:0,他引:1
Schröder JE Tegeler MR Grosshans U Porten E Blank M Lee J Esapa C Blake DJ Kröger S 《Developmental biology》2007,307(1):62-78
In the developing CNS alpha- and beta-dystroglycan are highly concentrated in the endfeet of radial neuroepithelial cells at the contact site to the basal lamina. We show that injection of anti-dystroglycan Fab fragments, knockdown of dystroglycan using RNAi, and overexpression of a dominant-negative dystroglycan protein by microelectroporation in neuroepithelial cells of the chick retina and optic tectum in vivo leads to the loss of their radial morphology, to hyperproliferation, to an increased number of postmitotic neurons, and to an altered distribution of several basally concentrated proteins. Moreover, these treatments also altered the oriented growth of axons from retinal ganglion cells and from tectal projection neurons. In contrast, expression of non-cleavable dystroglycan protein in neuroepithelial cells reduced their proliferation and their differentiation to postmitotic neurons. These results demonstrate that dystroglycan plays a key role in maintaining neuroepithelial cell morphology, and that interfering with dystroglycan function influences proliferation and differentiation of neuroepithelial cells. These data also suggest that an impaired dystroglycan function in neuroepithelial cells might be responsible for some of the severe brain abnormalities observed in certain forms of congenital muscular dystrophy. 相似文献