相关实验视频

Updated: Jan 18, 2026

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
07:14

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae

Published on: February 25, 2022

6.5K

基于蛋白质结构的FUS突变亚型与肌缩侧面硬化症患者的蛋白质错位化有关

Wanli Yang1,2,3, Zhen Luo4, Xuelin Tang1,2,3

  • 1Yangzhi Rehabilitation Hospital (Shanghai Sunshine Rehabilitation Centre), Clinical Center for Brain and Spinal Cord Research, School of Medicine, Tongji University, Shanghai, 200090, China.

Molecular neurobiology
|May 24, 2025
PubMed
概括

FUS基因的突变通过错误地定位RNA结合蛋白质,导致肌缩侧面硬化症 (ALS). 在FUS蛋白中的结构变化解释了ALS患者的疾病发病和严重程度的不同.

关键词:
发病时的年龄.肌缩性侧面硬化症 (AMLS) 是一种疾病.结合性亲缘关系是一种结合性亲缘关系.在瘤中融化.错位化错位化情况蛋白质结构 蛋白质结构

更多相关视频

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis08:59

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Published on: July 16, 2021

3.1K
Assay Development for High Content Quantification of Sod1 Mutant Protein Aggregate Formation in Living Cells11:12

Assay Development for High Content Quantification of Sod1 Mutant Protein Aggregate Formation in Living Cells

Published on: October 4, 2017

7.6K

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,...
11.7K
Amyloid Fibrils03:03

Amyloid Fibrils

6.3K
Protein Folding01:22

Protein Folding

Overview
126.5K
Protein Folding01:25

Protein Folding

Proteins are chains of amino acids linked together by peptide bonds. Upon synthesis, a protein folds into a three-dimensional conformation, critical to its biological function. Interactions between its constituent amino acids guide protein folding, and hence the protein structure is primarily dependent on its amino acid sequence.
Protein Structure Is Critical to Its Biological Function
Proteins perform a wide range of biological functions such as catalyzing chemical reactions, providing...
11.2K
Mutations01:39

Mutations

Overview
94.4K
Mutations01:35

Mutations

Mutations are changes in the sequence of DNA. These changes can occur spontaneously or they can be induced by exposure to environmental factors. Mutations can be characterized in a number of different ways: whether and how they alter the amino acid sequence of the protein, whether they occur over a small or large area of DNA, and whether they occur in somatic cells or germline cells.
Chromosomal Alterations Are Large-Scale Mutations
While point mutations are changes in a single nucleotide in...
43.0K