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関連する概念動画

Directing Proteins to the Rough Endoplasmic Reticulum01:34

Directing Proteins to the Rough Endoplasmic Reticulum

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The organelle-specific signaling sequences direct proteins synthesized in the cytosol to their final destination like ER, mitochondria, peroxisomes, etc. Some of the proteins directed to ER are then trafficked via vesicles to other organelles within the cell or the extracellular environment through the Golgi complex. For example, the rough ER synthesizes soluble proteins for transportation to the lysosomes or secretion out of the cell. It can also synthesize transmembrane proteins that can...
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Regulation of the Unfolded Protein Response01:31

Regulation of the Unfolded Protein Response

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Inositol-requiring kinase one or IRE1 is the most conserved eukaryotic unfolded protein response (UPR) receptor. It is a type I transmembrane protein kinase receptor with a distinctive site-specific RNase activity. As the binding mechanics of the misfolded proteins with the N-terminal domain of IRE-1 are unclear, three binding models — direct, indirect, and allosteric -- are proposed for receptor activation. Nevertheless, it is known that once a misfolded protein associates with IRE1, it...
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The Unfolded Protein Response01:37

The Unfolded Protein Response

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The ER is the hub of protein synthesis in a cell. It has robust systems to quality control protein folding and also for degradation of terminally misfolded proteins. Under normal conditions, a small proportion of misfolded proteins that cannot be salvaged need to be transported to the cytoplasm by the ER-associated degradation or ERAD pathways. However, if the ERAD cannot handle the misfolded proteins, the cell activates the unfolded protein response or UPR to adjust the protein folding...
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Rab Proteins01:14

Rab Proteins

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Rab proteins constitute the largest family of monomeric GTPases, of which 70 members are present in humans. Rab proteins and their effectors regulate consecutive stages of vesicle transport such as vesicle transport, docking, and fusion to the correct recipient membrane.
Rab proteins switch between a cytosolic, GDP-bound inactive state and a membrane-anchored, GTP-bound active state. By themselves, Rabs show slow rates of GDP/GTP exchange and GTP hydrolysis. Thus, Rab proteins are considered...
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Export of Misfolded Proteins out of the ER01:32

Export of Misfolded Proteins out of the ER

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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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MAPK Signaling Cascades01:07

MAPK Signaling Cascades

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Mitogen-activated protein kinase, or MAPK pathway, activates three sequential kinases to regulate cellular responses such as proliferation, differentiation, survival, and apoptosis. The canonical MAPK pathway starts with a mitogen or growth factor binding to an RTK. The activated RTKs stimulate Ras, which recruits Raf or MAP3 Kinase (MAPKKK), the first kinase of the MAPK signaling cascade. Raf further phosphorylates and activates MEK or MAP2 Kinases (MAPKK), which in turn phosphorylates MAP...
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Methods to Study Mrp4-containing Macromolecular Complexes in the Regulation of Fibroblast Migration
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Methods to Study Mrp4-containing Macromolecular Complexes in the Regulation of Fibroblast Migration

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スナップショット:FMRP相互作用タンパク質

Emanuela Pasciuto1, Claudia Bagni2

  • 1VIB Center for the Biology of Disease, 3000 Leuven, Belgium; Center for Human Genetics and Leuven Institute for Neurodegenerative Diseases (LIND), KU Leuven, 3000 Leuven, Belgium.

Cell
|September 27, 2014
PubMed
まとめ
この要約は機械生成です。

遺伝性知的障害や自閉症の一般的な原因である脆弱X症候群は,脆弱X精神障害タンパク質 (FMRP) の問題から生じる. この研究では,FMRPの相互作用パートナーと,細胞経路におけるそれらの役割について調べています.

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科学分野:

  • 神経遺伝学 神経遺伝学
  • 分子生物学は分子生物学である.
  • 発達神経科学とは

背景:

  • 脆いX症候群は,知的障害と自閉症スペクトラム障害の主要な遺伝的原因です.
  • 脆弱なX型精神障害タンパク質 (FMRP) は,この状態の病原性において中心的な役割を果たしています.
  • FMRPの分子機能を理解することは,治療戦略の開発に不可欠です.

研究 の 目的:

  • FMRPの既知のタンパク質相互作用パートナーの包括的な概要を提供するために.
  • FMRPによって調節される細胞経路と生物学的プロセスを解明する.
  • 脆弱X症候群の文脈におけるFMRP相互作用の重要性を強調する.

主な方法:

  • タンパク質とタンパク質の相互作用データベースの文献レビューとデータマイニング.
  • FMRPインタラクトームのバイオ情報分析.
  • 特定された相互作用パートナーの機能的エンリッチメント分析.

主要な成果:

  • FMRPと相互作用するタンパク質の多様なセットの識別.
  • これらのパートナーをRNA代謝,シナプス機能,信号伝導を含む重要な細胞経路に分類する.
  • ニューロンの発達と機能におけるFMRPの多面的な役割を強調する.

結論:

  • FMRPは,重要な細胞プロセスに関与する幅広い種類のタンパク質と相互作用します.
  • これらの相互作用は,脆弱X症候群の基礎にある複雑な分子機構を強調しています.
  • FMRPを媒介する経路をターゲットにすることで,知的障害や自閉症に対する潜在的な治療法を提供することができます.