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Related Concept Videos

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...
Alzheimer Disease ll: Pathophysiology01:23

Alzheimer Disease ll: Pathophysiology

Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
Long-patch Base Excision Repair01:02

Long-patch Base Excision Repair

Since the discovery of the two BER pathways, there has been a debate about how a cell chooses one pathway over the other and the factors determining this selection. Numerous in vitro experiments have pointed out multiple determinants for the sub-pathway selection. These are:
Lysosomal Hydrolases01:22

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...

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Biochemical Purification and Proteomic Characterization of Amyloid Fibril Cores from the Brain
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Protein clearing pathways in ALS.

Michela Ferrucci1, Fedrica Fulceri, Luca Toti

  • 1Department of Human Morphology and Applied Biology University of Pisa, Italy.

Archives Italiennes De Biologie
|March 18, 2011
PubMed
Summary

Defects in autophagy, a cellular cleaning process, are common in amyotrophic lateral sclerosis (ALS). Enhancing autophagy may offer new therapeutic strategies for motor neuron degeneration in ALS patients.

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Genetics

Background:

  • Protein clearing systems are vital for motor neuron survival, particularly during neurodegenerative conditions.
  • Autophagy, a key cellular mechanism, clears damaged organelles and misfolded proteins, promoting neuronal health.
  • Dysfunctional protein clearance contributes to neurodegeneration in disorders like amyotrophic lateral sclerosis (ALS).

Purpose of the Study:

  • To review experimental and clinical studies on amyotrophic lateral sclerosis (ALS).
  • To identify common pathogenic mechanisms in ALS, focusing on protein degradation pathways.
  • To explore the role of autophagy in motor neuron degeneration and its therapeutic potential for ALS.

Main Methods:

  • Review and synthesis of existing experimental and clinical research on ALS.
  • Analysis of studies investigating cellular protein clearing systems, with a focus on autophagy.
  • Examination of evidence linking autophagy defects to motor neuron pathology in ALS.

Main Results:

  • A defect in the autophagy machinery is a common finding in both genetic and sporadic ALS.
  • Accumulation of damaged mitochondria and protein aggregates within autophagosomes is observed in affected motor neurons.
  • Inhibition of autophagy accelerates motor neuron death and symptoms, while stimulation shows a protective effect.

Conclusions:

  • Autophagy plays a critical role in motor neuron survival and is commonly impaired in ALS.
  • Autophagy dysfunction contributes to the pathogenesis of amyotrophic lateral sclerosis.
  • Modulating autophagy presents a promising therapeutic target for treating ALS and other motor neuron disorders.