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Updated: Apr 5, 2026

Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells
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Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells

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C9orf72における GGGGCC 繰り返し膨張は,核細胞質輸送を危うくする

Brian D Freibaum1, Yubing Lu2, Rodrigo Lopez-Gonzalez2

  • 1Department of Cell and Molecular Biology, St Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.

Nature
|August 27, 2015
PubMed
まとめ

C9orf72のGGGGCC (G4C2) 繰り返し膨張は,核輸送を妨害することによって神経変性を引き起こす. この研究は,C9orf72に関連する疾患における重要なメカニズムとして核毛孔複合体の欠陥を特定しています.

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Export of Misfolded Proteins out of the ER

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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Lesson: Translation
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.
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