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Related Concept Videos

Proteins: From Genes to Degradation02:11

Proteins: From Genes to Degradation

Within a biological system, the DNA encodes the RNA, and the nucleotide sequence in the RNA further defines the amino acid sequence in the protein. This is referred to as “The Central Dogma of Molecular Biology” - a term coined by Francis Crick.  Central dogma is a firm principle in biology that defines the flow of genetic information within any life form. The two fundamental steps in central dogma are - transcription and translation.
Transcription is the synthesis of RNA molecules by RNA...
Proteins: From Genes to Degradation02:11

Proteins: From Genes to Degradation

Within a biological system, the DNA encodes the RNA, and the nucleotide sequence in the RNA further defines the amino acid sequence in the protein. This is referred to as “The Central Dogma of Molecular Biology” - a term coined by Francis Crick.  Central dogma is a firm principle in biology that defines the flow of genetic information within any life form. The two fundamental steps in central dogma are - transcription and translation.
Transcription is the synthesis of RNA molecules by RNA...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
The Proteasome01:13

The Proteasome

Eukaryotic cells can degrade proteins through several pathways. One of the most important among these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. This involves participation of a series of enzymes including— E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3 (ubiquitin...
The Proteasome02:18

The Proteasome

Eukaryotic cells can degrade proteins through several pathways. One of the most important amongst these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. A series of enzymes carry out the ubiquitination of the target proteins - E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...

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Assays for the Degradation of Misfolded Proteins in Cells
10:56

Assays for the Degradation of Misfolded Proteins in Cells

Published on: August 28, 2016

Protein degradation, aggregation, and misfolding.

Ana Maria Cuervo1, Esther S P Wong, Marta Martinez-Vicente

  • 1Department of Developmental and Molecular Biology, Marion Bessin Liver Research Center, Albert Einstein College of Medicine, Bronx, New York 10461, USA. amcuervo@aecom.yu.edu

Movement Disorders : Official Journal of the Movement Disorder Society
|February 27, 2010
PubMed
Summary

Cellular quality control systems remove damaged components. This review explores how their dysfunction, particularly in intracellular proteolytic systems, contributes to neurodegenerative diseases like Parkinson's disease.

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Last Updated: Jun 15, 2026

Assays for the Degradation of Misfolded Proteins in Cells
10:56

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Published on: August 28, 2016

Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans
09:18

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Published on: September 7, 2021

4D Imaging of Protein Aggregation in Live Cells
08:59

4D Imaging of Protein Aggregation in Live Cells

Published on: April 5, 2013

Area of Science:

  • Neurobiology
  • Cellular Biology
  • Molecular Biology

Background:

  • Cellular surveillance systems are crucial for maintaining cellular health by removing damaged components.
  • Dysfunction in these systems, especially in neurons, is implicated in the development of neurodegenerative disorders.
  • Intracellular proteolytic systems are key components of cellular quality control.

Purpose of the Study:

  • To review current understanding of how intracellular proteolytic systems contribute to neurodegeneration.
  • To highlight the role of cellular quality control system alterations in neurodegenerative pathogenesis.
  • To focus on the specific involvement of these systems in Parkinson's disease.

Main Methods:

  • Literature review of recent advances in cellular quality control and neurodegeneration research.
  • Analysis of the role of intracellular proteolytic systems in neuronal health.
  • Synthesis of evidence linking system alterations to neurodegenerative disease mechanisms.

Main Results:

  • Advances in understanding how impaired intracellular proteolytic systems lead to the accumulation of toxic proteins.
  • Evidence suggests a direct link between cellular quality control deficits and neurodegeneration.
  • Specific pathways within proteolytic systems are identified as critical in Parkinson's disease pathogenesis.

Conclusions:

  • Alterations in cellular surveillance and intracellular proteolytic systems are significant contributors to neurodegeneration.
  • Understanding these pathways offers potential therapeutic targets for neurodegenerative diseases.
  • Further research into these systems is vital for developing effective treatments for conditions like Parkinson's disease.