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The Ras Gene02:38

The Ras Gene

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The Ras-gene-encoded proteins are regulators of signaling pathways controlling cell proliferation, differentiation, or cell survival. The Ras-gene family in humans constitutes three primary members—the HRas, NRas, and KRas. These genes code for four functionally distinct yet closely related proteins—the HRas, NRas, KRas4A, and KRas4B. The involvement of mutant Ras genes in human cancer was first discovered in 1982 and is among the most common causes of human tumorigenesis.
Ras is a...
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Mutations01:39

Mutations

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Overview
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Mutations01:35

Mutations

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Mutations are changes in the sequence of DNA. These changes can occur spontaneously or they can be induced by exposure to environmental factors. Mutations can be characterized in a number of different ways: whether and how they alter the amino acid sequence of the protein, whether they occur over a small or large area of DNA, and whether they occur in somatic cells or germline cells.
Chromosomal Alterations Are Large-Scale Mutations
While point mutations are changes in a single nucleotide in...
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Viral Mutations00:36

Viral Mutations

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A mutation is a change in the sequence of bases of DNA or RNA in a genome. Some mutations occur during replication of the genome due to errors made by the polymerase enzymes that replicate DNA or RNA. Unlike DNA polymerase, RNA polymerase is prone to errors because it is not capable of “proofreading” its work. Viruses with RNA-based genomes, like HIV, therefore accrue mutations faster than viruses with DNA-based genomes. Because mutation and recombination provide the raw material...
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Small GTPases - Ras and Rho01:24

Small GTPases - Ras and Rho

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Ras and Rho are small monomeric GTPases that act downstream of receptor tyrosine kinase (RTK) and regulate various cellular processes. These GTPases switch between active and inactive states by binding to guanine nucleotides.
Three regulatory proteins control their activity:
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Energy to Drive Translocation01:37

Energy to Drive Translocation

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Mitochondrial protein import is powered by two distinct energy sources: ATP hydrolysis and electrochemical potential across the inner membrane. Newly synthesized precursors are bound by cytosolic chaperones of the Hsp70 family, which guide them to the import receptors on the mitochondrial surface. Utilizing the energy of ATP hydrolysis, Hsp70 chaperones transfer these precursors to the TOM receptors on the mitochondrial outer membrane.
Generally, polypeptides are unfolded by two distinct...
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Updated: Feb 2, 2026

In Vitro Ubiquitination and Deubiquitination Assays of Nucleosomal Histones
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In Vitro Ubiquitination and Deubiquitination Assays of Nucleosomal Histones

Published on: July 25, 2019

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LZTR1の変異は,RASのユビキチン化を乱すことでヒトの病気を誘発する.

M Steklov1,2, S Pandolfi1,2, M F Baietti1,2

  • 1VIB-KU Leuven Center for Cancer Biology, VIB, 3000 Leuven, Belgium.

Science (New York, N.Y.)
|November 17, 2018
PubMed
まとめ

レウシンジッパー型転写レギュレータ1 (LZTR1) タンパク質は,ユビキチネーションを媒介することによってRASシグナリングを調節する. LZTR1の機能の喪失はヌーナン症候群を引き起こし,シュヴァン細胞増殖に影響を与え,ヒトの病気におけるその役割を説明する.

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Differentiation, Maintenance, and Analysis of Human Retinal Pigment Epithelium Cells: A Disease-in-a-dish Model for BEST1 Mutations
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Differentiation, Maintenance, and Analysis of Human Retinal Pigment Epithelium Cells: A Disease-in-a-dish Model for BEST1 Mutations

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Detection of Protein Ubiquitination
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Detection of Protein Ubiquitination

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関連する実験動画

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Differentiation, Maintenance, and Analysis of Human Retinal Pigment Epithelium Cells: A Disease-in-a-dish Model for BEST1 Mutations
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Detection of Protein Ubiquitination
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Detection of Protein Ubiquitination

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科学分野:

  • 分子生物学
  • 遺伝学
  • 細胞生物学

背景:

  • レウシンジッパー型転写レギュレータ1 (LZTR1) タンパク質は,カルリン3 (CUL3) ユビキチンリガゼ複合体のアダプタです.
  • LZTR1の正確な作用機構とヒトの疾患における役割は,ほとんど不明である.

研究 の 目的:

  • LZTR1機能の分子メカニズムを解明する.
  • LZTR1,RASシグナル伝達と ヌーナン症候群のような人間の病気の関連を調べるため

主な方法:

  • 哺乳類の細胞から LZTR1複合体を捕まえる
  • タンパク質のユビキチン化変化を特定するためのユビキトーム分析
  • 病気に関連したLZTR1変異の分析

主要な成果:

  • LZTR1はCUL3ユビキチンリガゼ複合体のアダプタとして作用し,グアノシントリホスファタゼRASを標的とする.
  • LZTR1媒介によるRASのユビキチン化は,膜結合を減少させることでRASシグナル伝達を阻害する.
  • マウスにおけるLztr1の喪失は,ヌーナン症候群の表型を再現し,シュヴァン細胞の行動に影響する.
  • 特定された疾患関連LZTR1変異は複合体形成またはRAS相互作用を阻害する.

結論:

  • LZTR1媒介によるRASのユビキチネーションは重要な規制メカニズムである.
  • この経路の調節不良は,ヌーナン症候群を含むヒトの疾患におけるLZTR1の関与を説明する.