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Updated: May 5, 2026

Assays for the Degradation of Misfolded Proteins in Cells
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Assays for the Degradation of Misfolded Proteins in Cells

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ATAXIN-1的核定位和聚合:在SCA1转基因小鼠中,在多聚胺诱导的疾病中的作用

I A Klement1, P J Skinner, M D Kaytor

  • 1Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis 55455, USA.

Cell
|October 20, 1998
PubMed
概括

素-1的核定位对于小鼠1型脊髓小脑症 (SCA1) 发病的产生至关重要. 然而,核聚合的ATAXIN-1不需要启动疾病.

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Exogenous Administration of Microsomes-associated Alpha-synuclein Aggregates to Primary Neurons As a Powerful Cell Model of Fibrils Formation09:16

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相关概念视频

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
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