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Amyloid Fibrils03:03

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Alternative RNA Splicing02:18

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Alternative RNA splicing is the regulated splicing of exons and introns to produce different mature mRNAs from a single pre-mRNA. Unlike in constitutive splicing where a single gene produces a single type of mRNA, alternative splicing allows an organism to produce multiple proteins from a single gene and plays an important role in protein diversity.
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RNA editing is a post-transcriptional modification where a precursor mRNA (pre-mRNA) nucleotide sequence is changed by base insertion, deletion, or modification. The extent of RNA editing varies from a few hundred bases, in mitochondrial DNA of trypanosomes, to a just single base, in nuclear genes of mammals. Even a single base change in the pre-mRNA can convert a codon for one amino acid into the codon for another amino acid or a stop codon. This type of re-coding can significantly affect the...
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Unlike eukaryotes, bacteria use a single RNA Polymerase (RNAP) to transcribe all genes. The different subunits of bacterial RNAPhave distinct functions. The multisubunit structure of the bacterial RNAP helps the enzyme to maintain catalytic function, facilitate assembly, interact with DNA and RNA, and self-regulate its activity.
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RNA Polymerase (RNAP) is conserved in all animals, with bacterial, archaeal, and eukaryotic RNAPs sharing significant sequence, structural, and functional similarities. Among the three eukaryotic RNAPs, RNA Polymerase II is most similar to bacterial RNAP in terms of both structural organization and folding topologies of the enzyme subunits. However, these similarities are not reflected in their mechanism of action.
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TDP-43とRNAは,再生する筋肉でアミロイドのようなミオ粒子を形成する.

Thomas O Vogler1,2, Joshua R Wheeler2,3, Eric D Nguyen2,4

  • 1Department of Molecular, Cellular and Developmental Biology, University of Colorado, Boulder, CO, USA.

Nature
|November 23, 2018
PubMed
まとめ
この要約は機械生成です。

TDP-43タンパク質の細胞プラズマ集積は,神経筋疾患において一般的です. 研究者らは,正常な筋肉再生には,TDP-43が一時的な"ミオ粒"を形成することが含まれていることを発見しました.

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科学分野:

  • 生物化学
  • 分子生物学
  • 神経科学

背景:

  • ALSやインクルージョンボディミオパシーのような神経筋疾患の特徴です.
  • TARDBPの変異はTDP-43の結合を引き起こしますが,ほとんどの患者は未知のメカニズムを示唆するワイルドタイプのTDP-43の結合を持っています.

研究 の 目的:

  • 神経筋疾患における野生型TDP-43結合のメカニズムを調査する.
  • 骨格筋におけるTDP-43の正常な機能と,疾患の病原性におけるその潜在的な役割を調査する.

主な方法:

  • マウスとヒトのモデルにおける骨格筋再生におけるTDP-43の役割を研究した.
  • TDP-43を含む細胞質の集合体 (ミオ粒) を特徴づけている.
  • ミオ粒子の形成,mRNAの結合,クリアランス,およびアミロイド線維の発芽の可能性を評価した.

主要な成果:

  • TDP-43は骨格筋の形成に不可欠であり,再生中に一時的なアミロイドのような"ミオ粒"を形成します.
  • これらのミオ粒子は,サルコメリックタンパク質に対するmRNAを結合し,筋肉が成熟するにつれてクリアされます.
  • ミオ粒子はTDP-43アミロイド繊維をインビトロで発酵させることができ,インクルージョンボディミオパシーマウスモデルでは上昇します.

結論:

  • ミオ粒子は,筋肉の再生中に TDP - 43 の正常な,一時的な集合体を表します.
  • 常見の神経筋疾患における有毒なTDP-43アグレガートの主要な源は,ミオ粒子の組成またはクリアランスの不調である.