Jove
Visualize
联系我们
JoVE
x logofacebook logolinkedin logoyoutube logo
关于 JoVE
概览领导团队博客JoVE 帮助中心
作者
出版流程编辑委员会范围与政策同行评审常见问题投稿
图书馆员
用户评价订阅访问资源图书馆顾问委员会常见问题
研究
JoVE JournalMethods CollectionsJoVE Encyclopedia of Experiments存档
教育
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab Manual教师资源中心教师网站
使用条款与条件
隐私政策
政策

相关概念视频

Lysosomal Hydrolases01:22

Lysosomal Hydrolases

3.8K
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
3.8K
Translation01:31

Translation

15.0K
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of Life
Proteins are...
15.0K
Amyloid Fibrils03:03

Amyloid Fibrils

9.6K
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,...
9.6K

您也可能阅读

相关文章

通过共同作者、期刊和引用图与本文相关的文章。

排序
Same author

Juvenile and adult expression of polyglutamine expanded huntingtin produce distinct aggregate distributions in Drosophila muscle.

Human molecular genetics·2023
Same author

Expression of Heat Shock Protein 70 Is Insufficient To Extend <i>Drosophila melanogaster</i> Longevity.

G3 (Bethesda, Md.)·2019
查看所有相关文章

相关实验视频

Updated: Jul 17, 2025

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay
08:33

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay

Published on: June 25, 2019

8.3K

在外围组织中发生的多重质胺疾病.

Taylor Barwell1, Laurent Seroude1

  • 1Department of Biology, Queen's University, 116 Barrie St, Kingston, ON K7L 3N6, Canada.

Human molecular genetics
|August 29, 2023
PubMed
概括
此摘要是机器生成的。

亨廷顿病 (HD) 与亨廷丁基因有关. 新的研究表明,突变的亨廷丁在外围组织中,而不仅仅是大脑,显着加剧了HD症状.

科学领域:

  • 神经科学是一个神经科学.
  • 遗传学 遗传学 是一个
关键词:
亨廷顿病就是亨廷顿病.狩猎 狩猎 狩猎 狩猎模型生物模型生物.蛋白质聚合物是一种蛋白质聚合物.

更多相关视频

Growth Assays to Assess Polyglutamine Toxicity in Yeast
09:06

Growth Assays to Assess Polyglutamine Toxicity in Yeast

Published on: March 5, 2012

13.6K
Caenorhabditis elegans as a Model System for Discovering Bioactive Compounds Against Polyglutamine-Mediated Neurotoxicity
08:16

Caenorhabditis elegans as a Model System for Discovering Bioactive Compounds Against Polyglutamine-Mediated Neurotoxicity

Published on: September 21, 2021

3.4K

相关实验视频

Last Updated: Jul 17, 2025

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay
08:33

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay

Published on: June 25, 2019

8.3K
Growth Assays to Assess Polyglutamine Toxicity in Yeast
09:06

Growth Assays to Assess Polyglutamine Toxicity in Yeast

Published on: March 5, 2012

13.6K
Caenorhabditis elegans as a Model System for Discovering Bioactive Compounds Against Polyglutamine-Mediated Neurotoxicity
08:16

Caenorhabditis elegans as a Model System for Discovering Bioactive Compounds Against Polyglutamine-Mediated Neurotoxicity

Published on: September 21, 2021

3.4K
  • 细胞生物学 细胞生物学
  • 背景情况:

    • 亨廷顿氏病 (HD) 是由于亨廷丁基因中的扩大多重胺重复引起的,导致蛋白质聚合.
    • 尽管HD的发现已经过去了30年,但它仍然难以治愈,目前的治疗方法有不良影响.
    • 亨廷丁基因在许多非神经系统组织中表达,但它们在疾病发病过程中的作用一直被忽视.

    结论:

    • 外围亨廷丁基因表达和聚合物形成有助于亨廷顿病.
    • 了解这些非神经元作用对于开发新的治疗策略至关重要.
    • 针对外围组织可能为亨廷顿病提供新的治疗途径.