排序方式: 共有97条查询结果,搜索用时 15 毫秒
91.
《Structure (London, England : 1993)》2023,31(6):644-650.e5
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92.
Ramita Dewan Ruth Chia Jinhui Ding Richard A. Hickman Thor D. Stein Yevgeniya Abramzon Sarah Ahmed Marya S. Sabir Makayla K. Portley Arianna Tucci Kristina Ibáñez F.N.U. Shankaracharya Pamela Keagle Giacomina Rossi Paola Caroppo Fabrizio Tagliavini Maria L. Waldo Per M. Johansson Bryan J. Traynor 《Neuron》2021,109(3):448-460.e4
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93.
《Expert review of proteomics》2013,10(1):17-20
The National Cancer Institute of the US National Institutes of Health established a Clinical Proteomic Technologies Initiative for Cancer (CPTI) in 2006. The first annual meeting organized by the CPTI program provided up-to-date information on the research activities and achievements at its first anniversary of this program. Presentations were made by leaders from the five centers nationwide of the Clinical Proteomic Technology Assessment for Cancer (CPTAC), and other principal investigators funded by the CPTI. 相似文献
94.
Expansion of CAG trinucleotide repeat within the coding region of several genes results in the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of these pathogenic proteins leads to PolyQ diseases, such as Huntington's disease or several types of spinocerebellar ataxias. This family of neurodegenerative disorders is characterized by constant progression of the symptoms and molecularly, by the accumulation of mutant proteins inside neurons causing their dysfunction and eventually death. So far, no effective therapy actually preventing the physical and/or mental decline has been developed. Experimental therapeutic strategies either target the levels or processing of mutant proteins in an attempt to prevent cellular deterioration, or they are aimed at the downstream pathologic effects to reverse or ameliorate the caused damages. Certain pathomechanistic aspects of PolyQ disorders are discussed here. Relevance of disease models and recent knowledge of therapeutic possibilities is reviewed and updated. 相似文献
95.
《Cell reports》2023,42(1):111953
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96.
97.
Erin B.D. Clabough 《The Yale journal of biology and medicine》2013,86(2):217-233
Huntington’s disease (HD) is an autosomal dominant genetic disorder that
specifically causes neurodegeneration of striatal neurons, resulting in a triad
of symptoms that includes emotional, cognitive, and motor disturbances. The HD
mutation causes a polyglutamine repeat expansion within the N-terminal of the
huntingtin (Htt) protein. This expansion causes aggregate formation within the
cytosol and nucleus due to the presence of misfolded mutant Htt, as well as
altered interactions with Htt’s multiple binding partners, and changes in
post-translational Htt modifications. The present review charts efforts toward a
therapy that delays age of onset or slows symptom progression in patients
affected by HD, as there is currently no effective treatment. Although silencing
Htt expression appears promising as a disease modifying treatment, it should be
attempted with caution in light of Htt’s essential roles in neural maintenance
and development. Other therapeutic targets include those that boost aggregate
dissolution, target excitotoxicity and metabolic issues, and supplement growth
factors. 相似文献