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Related Concept Videos

The Ras Gene02:38

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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.
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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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Related Experiment Video

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In Vitro Ubiquitination and Deubiquitination Assays of Nucleosomal Histones
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Mutations in LZTR1 drive human disease by dysregulating RAS ubiquitination.

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
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Summary
This summary is machine-generated.

Leucine zipper-like transcriptional regulator 1 (LZTR1) protein regulates RAS signaling by mediating ubiquitination. Loss of LZTR1 function causes Noonan syndrome and affects Schwann cell proliferation, explaining its role in human disease.

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Area of Science:

  • Molecular Biology
  • Genetics
  • Cell Biology

Background:

  • The leucine zipper-like transcriptional regulator 1 (LZTR1) protein is an adaptor for the cullin 3 (CUL3) ubiquitin ligase complex.
  • LZTR1's precise mechanism of action and its role in human diseases remain largely unknown.

Purpose of the Study:

  • To elucidate the molecular mechanism of LZTR1 function.
  • To investigate the link between LZTR1, RAS signaling, and human diseases like Noonan syndrome.

Main Methods:

  • Trapping LZTR1 complexes from mammalian cells.
  • Ubiquitome analysis to identify protein ubiquitination changes.
  • Analysis of disease-associated LZTR1 mutations.

Main Results:

  • LZTR1 acts as an adaptor for the CUL3 ubiquitin ligase complex, targeting the guanosine triphosphatase RAS.
  • LZTR1-mediated ubiquitination of RAS at lysine-170 inhibits RAS signaling by reducing membrane association.
  • Loss of Lztr1 in mice recapitulates Noonan syndrome phenotypes and affects Schwann cell behavior.
  • Identified disease-associated LZTR1 mutations impair complex formation or RAS interaction.

Conclusions:

  • LZTR1-mediated ubiquitination of RAS is a key regulatory mechanism.
  • Dysregulation of this pathway explains LZTR1's involvement in human diseases, including Noonan syndrome.