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Related Concept Videos

Quality Control01:05

Quality Control

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Quality control is one of the three cyclical quality assurance activities that help keep a system under statistical control. Typical quality control activities include creating quality control charts, conducting proficiency testing, and documenting and archiving results.
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Regulated Protein Degradation02:58

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It is vital to regulate the activity of enzymatic as well as non-enzymatic proteins inside the cell. This can be achieved either through creating a balance between their rate of synthesis and degradation or regulating the intrinsic activity of the protein. Both these regulation mechanisms play an essential role in the normal functioning of cells.
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Master transcription regulators are regulatory proteins that are predominantly responsible for regulating the expression of multiple genes. Often these genes work in concert to drive a  complex process. Activation of a master transcription regulator can lead to a cascade of transcriptional activation necessary for that outcome. These regulators can directly bind to the regulatory sequences of the various genes involved, or they can indirectly regulate transcription by binding to regulatory...
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GTPases and their Regulation02:14

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Quality of Water01:19

Quality of Water

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In concrete preparation, the quality of water is paramount as it affects the strength and durability of the concrete. Potable water is usually preferred; however, it must not have excessive sodium or potassium to prevent compromising the concrete's integrity. Water quality is typically evaluated based on impurities such as dissolved solids, chlorides, and sulfates, and its pH value is ideally between 6 and 8. Even slightly acidic natural water may be acceptable unless it contains harmful...
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Related Experiment Video

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Studying the Protein Quality Control System of D. discoideum Using Temperature-controlled Live Cell Imaging
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L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control.

Jiayin Lu1,2, Goran Periz1,2, Yu-Ning Lu1,2

  • 1Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, USA.

Nature Neuroscience
|May 8, 2019
PubMed
Summary

Lethal(3)malignant brain tumor-like protein 1 (L3MBTL1) regulates protein quality control, protecting against neurodegenerative disease. Its loss shields neurons from toxic protein buildup, offering therapeutic potential for conditions like ALS and FTD.

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Area of Science:

  • Neurobiology
  • Molecular Biology
  • Genetics

Background:

  • Misfolded protein accumulation and impaired protein quality control are hallmarks of neurodegenerative diseases like ALS and FTD.
  • Identifying key regulators of protein quality control is crucial for understanding disease mechanisms.

Purpose of the Study:

  • To identify novel regulators of protein quality control involved in neurodegeneration.
  • To elucidate the role of Lethal(3)malignant brain tumor-like protein 1 (L3MBTL1) in protein quality control and neuroprotection.

Main Methods:

  • Proteotoxicity assays using mutant proteins (SOD1, C9orf72 repeats).
  • Analysis of p53-dependent protein degradation pathways.
  • Western blotting and immunohistochemistry in cell and animal models.
  • Comparative analysis across species (C. elegans to mammals).

Main Results:

  • L3MBTL1 was identified as a key regulator of protein quality control.
  • Loss of L3MBTL1 conferred protection against proteotoxicity from mutant SOD1 and C9orf72 proteins.
  • L3MBTL1 regulates p53-dependent degradation of misfolded proteins.
  • SET domain-containing protein 8, an L3MBTL1-associated protein, also impacts protein clearance.
  • L3MBTL1 and SETDB1 are upregulated in ALS/FTD models and patients.
  • L3MBTL1's function in protein quality control is evolutionarily conserved.

Conclusions:

  • A novel protein quality control pathway involving L3MBTL1 and SETDB1 is revealed.
  • This pathway is critical for cellular stress response and implicated in proteotoxicity-driven neurodegenerative diseases.
  • Targeting this pathway may offer therapeutic strategies for ALS and FTD.