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Delivery Pathways to the Lysosome01:36

Delivery Pathways to the Lysosome

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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.
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Autophagy is a self-digesting process by which a cell protects itself from threats both within and outside the cell, ranging from abnormal proteins to invading bacteria. In this process, obsolete components of the cell and invading microbes are degraded by hydrolytic enzymes active in an acidic environment of the lysosomal lumen.
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Christian de Duve discovered “autophagy,” a process in which cellular components are engulfed by membrane-bound organelles called autophagosomes. The autophagosomes then fuse with lysosomes to digest the enclosed contents. Autophagy is generally activated in cells to prevent cell death. However, cell death is triggered when the damage is beyond repair.
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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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Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles
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ポリグルタミン経路はベクリン1依存の自食を調節する

Avraham Ashkenazi1, Carla F Bento1, Thomas Ricketts1

  • 1Department of Medical Genetics, Cambridge Institute for Medical Research (CIMR), University of Cambridge, Cambridge, UK.

Nature
|April 27, 2017
PubMed
まとめ

タンパク質のポリグルタミン (polyQ) 経路の拡張は神経変性疾患を引き起こす. この研究は,病気を引き起こすポリQ膨張によって機能が妨げられるベクリン1を安定させることで,野生型のポリQ経路がオートファギーを可能にすることを明らかにしています.

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科学分野:

  • 神経生物学
  • 分子生物学
  • 遺伝学

背景:

  • 9つの神経変性疾患は,タンパク質のポリグルタミン (polyQ) 経路の拡張と関連しています.
  • PolyQの膨張は病気の発症年齢を減らし,溶解性タンパク質の形態でも有毒である.
  • 細胞質タンパク質における正常なポリQ経路の正確な機能は,ほとんど不明である.

研究 の 目的:

  • デウビキチン化酵素アタクシン3におけるポリQドメインの機能を明らかにする.
  • ポリQ経路の長さがタンパク質の相互作用と細胞プロセスにどのように影響するか調査する.
  • ポリQの拡張が神経変性疾患の病原化に寄与するメカニズムを理解する.

主な方法:

  • 細胞と体内のモデルを用いて野生型アタキシン3とベクリン1の相互作用を調査した.
  • ヒトの細胞系とマウスのニューロンにおける飢餓誘発の自閉症に対するアタキシン3の減少の影響を評価した.
  • 異なる長さのポリQ経路とそのオートファギーの相互作用を調査した.

主要な成果:

  • 野生型アタキシン3のポリQドメインは,ベクリン1との相互作用を促進し,ベクリン1の分解を防止します.
  • 細胞および動物モデルでは,アタキシン3の枯渇が飢餓誘発の自を有意に抑制した.
  • 病気のタンパク質におけるより長いポリQ経路は,ワイルド型アタキシン3と競合し,ハンチントン病のモデルと患者の細胞における自閉症を阻害した.

結論:

  • 野生型のポリQ管は,ベクリンとの相互作用によって,自己消化を維持する上で重要な役割を果たします.
  • 病気に関連したポリQ膨張は,野生型タンパク質と競合することで,この重要な機能を妨害し,オートファギーの障害を引き起こす.
  • このメカニズムは,神経変性疾患におけるポリQ膨張の新しい非集積的病原性機能を強調する.