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ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

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In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased...
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ATP Synthase: Structure01:18

ATP Synthase: Structure

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ATP synthase or ATPase is among the most conserved proteins found in bacteria, mammals, and plants. This enzyme can catalyze a forward reaction in response to the electrochemical gradient, producing ATP from ADP and inorganic phosphate. ATP synthase can also work in a reverse direction by hydrolyzing ATP and generating an electrochemical gradient. Different forms of ATP synthases have evolved special features to meet the specific demands of the cell. Based on their specific feature, ATP...
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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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Protein Import into the Peroxisomes01:27

Protein Import into the Peroxisomes

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Cells contain membrane-bound organelles called peroxisomes that oxidize organic molecules by transferring hydrogen atoms to oxygen, producing hydrogen peroxide. Peroxisomes enzymatically convert the released hydrogen peroxide into water and oxygen.
Peroxisomal Protein Import:
Peroxisomes lack the genetic machinery required to code for their own proteins. Hence, most peroxisomal membrane, lumenal and transmembrane proteins are synthesized in the cytoplasm or ER and transported to the peroxisome...
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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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Translation01:31

Translation

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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...
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ATP13A2の欠乏はリゾソームポリアミンの輸出を妨害する

Sarah van Veen1, Shaun Martin1, Chris Van den Haute2,3

  • 1Laboratory of Cellular Transport Systems, Department of Cellular and Molecular Medicine, KU Leuven, Leuven, Belgium.

Nature
|January 31, 2020
PubMed
まとめ

ATP13A2は,細胞の健康に不可欠なリゾソームポリアミンエクスポーターとして特定されています. パーキンソン病のような 神経退行性疾患に繋がる 機能障害により ポリアミンの輸送が妨げられ 細胞が死に至ります

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Targeted Next-generation Sequencing and Bioinformatics Pipeline to Evaluate Genetic Determinants of Constitutional Disease
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科学分野:

  • 神経科学
  • 細胞生物学
  • 遺伝学

背景:

  • ATP13A2 (PARK9) は,クフォール・ラケブ症候群や早期発症のパーキンソン病などの神経変性疾患に関連した内分泌体トランスポーターです.
  • ATP13A2はパーキンソン病の危険因子から保護し,その喪失はリソソームを損なうが,リソソーム内の特定の輸送機能は不明である.
  • ATP13A2の役割を理解することは,神経変性およびリソソーム機能不全のメカニズムを解明するために重要です.

研究 の 目的:

  • 溶解体内のATP13A2の輸送機能を決定する.
  • ポリアミン輸送と細胞毒性における ATP13A2の役割を調査する.
  • 欠陥のあるリソソミカルポリアミンの輸出と神経変性との関係を確立する.

主な方法:

  • ポリアミンの結合と輸送運動を評価するために,浄化されたATP13A2を用いた生化学的測定法.
  • 神経変性疾患に関連するATP13A2変異体の機能研究
  • 細胞吸収実験は,内細胞とリソソーム経路によるポリアミン輸送を測定します.
  • ポリアミン濃度とATP13A2発現に反応する細胞毒性,リソソーム完全性,およびキャセプシンB活性化の分析.
  • ATP13A2またはそのオートロジーを発現するニューロンおよびネマトードにおけるin vivo研究.

主要な成果:

  • ATP13A2は,精子に対する高い親和性を持つリゾソーマポリアミンエクスポーターとして機能する.
  • ポリアミンは精製されたATP13A2活性を刺激し,病気に関連したATP13A2変異体は,疾患の重度に関連した機能障害を示します.
  • ATP13A2は,細胞内のポリアミンの吸収をエンドサイトーシス経由で促進し,その後の細胞溶液への輸送を促し,細胞内のポリアミンの獲得におけるエンドリゾームの役割を示唆する.
  • 高濃度のポリアミンは細胞毒性を誘発し,ATP13A2の損失により悪化し,リソソーム機能障害,破裂,およびカセプシンBの活性化につながる.
  • 神経細胞と線虫のATP13A2発現障害は,観察された毒性フェノタイプを再現する.

結論:

  • ATP13A2による不完全なリゾソームポリアミンの輸出は,リゾソーム依存の細胞死亡のメカニズムとして特定されています.
  • このメカニズムは神経変性疾患の病原化に寄与する可能性があります.
  • この研究は,哺乳類のポリアミン輸送システムの分子的アイデンティティを明らかにし,細胞ポリアミンホメオスタシスとその疾患における障害に関する新しい洞察を提供します.