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相关概念视频

Lysosomal Hydrolases01:22

Lysosomal Hydrolases

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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,...
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Delivery Pathways to the Lysosome01:36

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Eukaryotic cells use different mechanisms to eliminate toxic waste obsolete and worn-out substances. Lysosomes play a pivotal role in this, and hence, these substances are carried to the lysosome from other parts of the cell and extracellular space through different pathways. The most elaborately studied pathways to the lysosome are the endocytic pathways.
Endocytosis
In endocytosis, the cell membrane takes up macromolecules and particles from the surrounding medium. Clathrin-mediated...
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Recycling Endosomes and Transcytosis00:58

Recycling Endosomes and Transcytosis

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The recycling endosome, also known as the endosomal recycling compartment (ERC), is a part of the slow-recycling process of the endocytic pathway. Molecules internalized through receptor-mediated endocytosis are either degraded in the lysosomes or are recycled to the plasma membrane through the fast- or slow-recycling route.
The recycling endosome is not a single organelle but an extensively tubulated network of recycling pathways. It functions in storing molecules or transporting them across...
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The Early Endosome: Endocytosis of Transferrin01:28

The Early Endosome: Endocytosis of Transferrin

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Essential proteins such as insulin or low-density lipoprotein (LDL) and micronutrients such as iron enter a eukaryotic cell through receptor-mediated endocytosis. Subsequently, the early endosomes fuse with the vesicles containing such receptor-ligand complexes and play a vital role in sorting the incoming ligands and receptors. While the ligands are either degraded inside the vesicle or released into the cytosol, their receptors are returned to the plasma membrane for further rounds of...
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Export of Misfolded Proteins out of the ER01:32

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After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
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The Proteasome01:13

The Proteasome

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Eukaryotic cells can degrade proteins through several pathways. One of the most important among these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
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相关实验视频

Updated: Jul 13, 2025

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内解酶体通路和ALS/FTD

Tiffany W Todd1, Wei Shao1, Yong-Jie Zhang2

  • 1Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.

Trends in neurosciences
|October 12, 2023
PubMed
概括
此摘要是机器生成的。

肌缩侧面硬化症 (ALS) 和前性痴呆症 (FTD) 与内分泌体系统有关. 破坏这个系统,而不仅仅是自,可能是ALS和FTD进展的关键.

关键词:
C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C9ORF72C在TDP-43中使用.在TMEM106B中使用.自自是自的过程.神经退行症的神经退行症蛋白质病变是一种蛋白质病变.

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科学领域:

  • 神经科学是一个神经科学.
  • 遗传学 遗传学 是一个
  • 细胞生物学 细胞生物学

背景情况:

  • 肌缩侧面硬化症 (ALS) 和前性痴呆症 (FTD) 是神经退行性疾病,具有重叠的遗传联系.
  • 涉及到ALS/FTD的几种基因与内溶性体路径有关.
  • 人们越来越认识到内分泌体功能障碍在ALS/FTD发病过程中的作用.

研究的目的:

  • 审查ALS/FTD与内分泌体系统之间的复杂联系.
  • 阐明与疾病相关的基因如何影响内分泌体路径.
  • 为了探索内分泌体中断的下游后果,超越了自.

主要方法:

  • 文献综述和当前研究结果的综合.
  • 在ALS/FTD中分析与内解体系统的遗传关联.
  • 讨论涉及内分泌体功能障碍的致病机制.

主要成果:

  • 有证据表明,ALS/FTD与内分泌体系统之间存在很强的联系.
  • 与ALS/FTD相关的特定基因直接影响内分泌体功能.
  • 内分泌体系统的干扰,而不是仅仅是自功能障碍,可能会导致疾病病理.

结论:

  • 内分泌体系统是ALS和FTD病变发生过程中的关键参与者.
  • 准内分泌体功能障碍为ALS/FTD提供了潜在的治疗途径.
  • 需要进一步研究内分泌干扰的非自后果.