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The Periodic Table03:25

The Periodic Table

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As early chemists discovered more elements, they realized that various elements could be grouped by their similar chemical behaviors. One such grouping includes lithium (Li), sodium (Na), and potassium (K). All of these elements are shiny, conduct heat and electricity well, and have similar chemical properties. A second grouping includes calcium (Ca), strontium (Sr), and barium (Ba), which also are shiny, good conductors of heat and electricity, and have chemical properties in common. However,...
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Periodic Classification of the Elements04:00

Periodic Classification of the Elements

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The periodic table arranges atoms based on increasing atomic number so that elements with the same chemical properties recur periodically. When their electron configurations are added to the table, a periodic recurrence of similar electron configurations in the outer shells of these elements is observed. Because they are in the outer shells of an atom, valence electrons play the most important role in chemical reactions. The outer electrons have the highest energy of the electrons in an atom...
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Pore Size Distribution01:23

Pore Size Distribution

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In concrete, the pore size distribution significantly influences the material's properties. Capillary pores, markedly larger than gel pores, form a vast network within partially hydrated cement paste, reducing the concrete's strength and increasing its permeability. This heightened permeability leads to a greater risk of damage from environmental factors like freeze-thaw cycles and chemical attacks, with the extent of vulnerability also being tied to the water-to-cement ratio.
Adequate...
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Underflow Gates

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Underflow gates are vital for controlling water flow in irrigation canals. The three main types of underflow gates — vertical, radial, and drum gates — serve different purposes while ensuring effective flow management. Vertical gates move up and down, generating a free-flowing water jet; radial gates pivot to regulate the flow; and drum gates rotate for precise adjustments. The flow through these gates is influenced by downstream conditions, resulting in free or drowned outflow.Free and...
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Ligand-gated ion channels are transmembrane proteins that play a vital role in intercellular communication and functions of the nervous system. They allow the influx of ions across the membrane once the neurotransmitter binds, allowing the subsequent transmission of electrical excitation across the neurons. Other ligand-gated ion channels, like the γ-aminobutyric acid (GABA) receptor, permit anions like chloride into the cells on the binding of the GABA molecule. Their entry into the cell...
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Effective Value of a Periodic Waveform01:07

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The concept of effective value, the root mean square (RMS) value, is crucial in understanding electrical circuits and power delivery. This idea emerges from the necessity to measure the effectiveness of a voltage or current source in supplying power to a resistive load.
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周期性麻痺における穴の流れの構造的基礎

Daohua Jiang1, Tamer M Gamal El-Din1, Christopher Ing2,3

  • 1Department of Pharmacology, University of Washington, Seattle, WA, USA.

Nature
|May 18, 2018
PubMed
まとめ

イオンチャネルの変異は 周期的な麻痺を引き起こし 漏れを引き起こします 構造的研究により 遺伝性疾患の筋肉の弱さを治療する薬の標的を特定する 原子的メカニズムが明らかになりました

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科学分野:

  • 分子生物学
  • バイオ物理学
  • 遺伝学

背景:

  • 周期性麻痺は,エピソード的な筋肉の弱さを引き起こす遺伝的な骨格筋疾患を含む.
  • これらの条件は,電圧ゲートされたナトリウム (Nav1.4) またはカルシウム (Cav1.1) チャンネルの変異から生じる.
  • ゲーティングチャージ (R1-R3) の特定の変異は,カチオン漏れによる低カリミアまたはノルコカリミア形態につながる.

研究 の 目的:

  • 周期性麻痺の原子レベルのメカニズムを解明する
  • 電圧ゲートイオンチャネルにおけるカチオン漏れの構造的基礎を調査する.
  • 治療の標的となる薬物を特定する.

主な方法:

  • 変異した細菌のナトリウムチャンネルNavAbの高解像度構造分析
  • 分子モデリングとシミュレーション
  • グアニジニウムと複合したNavAb(R2Gの結晶学.

主要な成果:

  • NavAbのR2GとR3Gの変異は,電圧センサーのバックボーン構造を変化させなかった.
  • これらの変異は水性穴を作り カチオン漏れを容易にした.
  • R3Gは活性化状態で連続したトランスメブラン経路を可能にし,R2Gは静止状態で1つを生み出しました.
  • NavAb(R2G) のグアニジニウム結合部位は,薬物の標的を示唆している.

結論:

  • ゲート孔の形成に関する構造的な洞察は 周期性麻痺の病原性を明らかにする.
  • 発見はイオンチャネル機能不全の 原子レベルのメカニズムを明らかにします
  • 周期性麻痺の症状緩和のための潜在的な薬標的が特定されました.