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Weili Yang
Jinan University
Cas9NeurodegenerationGeneticsBiologyCRISPR
15Publications
8H-index
407Citations
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Publications 15
Newest
#1Peng Yin (Emory University)H-Index: 6
#2Qiong Liu (CSU: Central South University)H-Index: 1
Last. Xiao-Jiang Li (JNU: Jinan University)H-Index: 1
view all 11 authors...
Demyelination is a common pathological feature of a large number of neurodegenerative diseases including multiple sclerosis and Huntington's disease (HD). Laquinimod (LAQ) has been found to have therapeutic effects on multiple sclerosis and HD. However, the mechanism underlying LAQ's therapeutic effects remains unknown. Using HD mice that selectively express mutant huntingtin in oligodendrocytes and show demyelination, we found that LAQ reduces the Ser259 phosphorylation on myelin regulatory fac...
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#1Xingxing Chen (WUST: Wuhan University of Science and Technology)
#2Ning XinH-Index: 1
Last. Xiao-Jiang LiH-Index: 65
view all 10 authors...
Huntingtin-associated protein 1 (Hap1) was initially identified as a brain-enriched protein that binds to the Huntington's disease protein, huntingtin. Unlike huntingtin that is ubiquitously expressed in the brain, Hap1 is enriched in the brain with the highest expression level in the hypothalamus. The selective enrichment of Hap1 in the hypothalamus suggests that Hap1 may play a specific role in hypothalamic function that can regulate metabolism and stress response. Here we report that Hap1 is ...
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#1Huiming YangH-Index: 3
#2Su Yang (JNU: Jinan University)H-Index: 1
Last. Zhaohui S. Qin (Emory University)H-Index: 45
view all 12 authors...
Polyglutamine expansion in proteins can cause selective neurodegeneration, although the mechanisms are not fully understood. In Huntington’s disease (HD), proteolytic processing generates toxic N-terminal huntingtin (HTT) fragments that preferentially kill striatal neurons. Here, using CRISPR/Cas9 to truncate full-length mutant HTT in HD140Q knock-in (KI) mice, we show that exon 1 HTT is stably present in the brain, regardless of truncation sites in full-length HTT. This N-terminal HTT leads to ...
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#1Weili Yang (Emory University)H-Index: 1
#2Li Shihua (Emory University)H-Index: 5
Last. Xiao-Jiang Li (Emory University)H-Index: 65
view all 3 authors...
Genetically modified rodent models have been valuable for investigating the pathogenesis of neurodegenerative diseases such as Parkinson’s disease (PD). Based on the fact that mutations in the PINK1 gene cause autosomal recessive juvenile parkinsonism, a number of mouse models with deletion of the PINK1 gene were generated. However, these PINK1 knockout mouse models fail to recapitulate the selective and overt neurodegeneration seen in PD patient brains. Recently, we generated a non-human primat...
2 CitationsSource
#1Peng Yin (JNU: Jinan University)H-Index: 1
#1Peng Yin (Emory University)H-Index: 6
Last. Xiao-Jiang Li (Emory University)H-Index: 65
view all 10 authors...
The cytoplasmic accumulation of the nuclear TAR DNA-binding protein 43 (TDP-43) is a pathologic hallmark in amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and other neurological disorders. However, most transgenic TDP-43 rodent models show predominant nuclear distribution of TDP-43 in the brain. By expressing mutant TDP-43 (M337V) in the brains of rhesus monkeys and mice, we verified that mutant TDP-43 is distributed in the cytoplasm of the monkey brain and that the majority o...
6 CitationsSource
#1Weili Yang (JNU: Jinan University)H-Index: 8
#2Yunbo Liu (Peking Union Medical College)H-Index: 2
Last. Xiao-Jiang Li (Emory University)H-Index: 65
view all 15 authors...
8 CitationsSource
#1Sen Yan (JNU: Jinan University)H-Index: 9
#2Zhuchi Tu (JNU: Jinan University)H-Index: 8
Last. Xiao-Jiang Li (Emory University)H-Index: 65
view all 21 authors...
Summary Huntington's disease (HD) is characterized by preferential loss of the medium spiny neurons in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we established a knockin (KI) pig model of HD that endogenously expresses full-length mutant huntingtin (HTT). By breeding this HD pig model, we have successfully obtained F1 and F2 generation KI pigs. Characterization of founder and F1 KI pigs shows consistent movement, behavioral abnormalities, and early death, which are...
63 CitationsSource
#1Su Yang (Emory University)H-Index: 11
#2Huiming Yang (CSU: Central South University)H-Index: 3
Last. Xiao-Jiang Li (Emory University)H-Index: 65
view all 10 authors...
The hypothalamus has a vital role in controlling food intake and energy homeostasis; its activity is modulated by neuropeptides and endocrine factors. Mesencephalic astrocyte-derived neurotrophic factor (MANF) is a neurotrophic factor that is also localized in the endoplasmic reticulum (ER) in neurons. Here we show that MANF is highly enriched in distinct nuclei of the mouse hypothalamus, and that MANF expression in the hypothalamus is upregulated in response to fasting. Increasing or decreasing...
19 CitationsSource
#1Xiao-Jiang Li (Emory University)H-Index: 65
#2Zhuchi Tu (JNU: Jinan University)H-Index: 8
Last. Li Shihua (Emory University)H-Index: 5
view all 4 authors...
Off-target effects and mosaicism are major concerns for applying CRISPR-Cas9 to correct genetic mutations. A recent article in Nature by Ma et al. (2017) uses an elegant CRISPR-Cas9 approach that repairs a genetic mutation in human embryos with negligible mosaicism and no off-target effects, bringing this editing tool closer to clinical application.
2 CitationsSource
#1Zhuchi Tu (CAS: Chinese Academy of Sciences)H-Index: 8
#2Weili Yang (CAS: Chinese Academy of Sciences)H-Index: 8
Last. Xiao-Jiang LiH-Index: 65
view all 13 authors...
CRISPR-Cas9 is a powerful new tool for genome editing, but this technique creates mosaic mutations that affect the efficiency and precision of its ability to edit the genome. Reducing mosaic mutations is particularly important for gene therapy and precision genome editing. Although the mechanisms underlying the CRSIPR/Cas9-mediated mosaic mutations remain elusive, the prolonged expression and activity of Cas9 in embryos could contribute to mosaicism in DNA mutations. Here we report that tagging ...
41 CitationsSource
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