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Related Concept Videos

Autophagy01:27

Autophagy

Autophagy is a self-digesting process by which a cell protects itself from threats both within and outside the cell, ranging from abnormal proteins to invading bacteria. In this process, obsolete components of the cell and invading microbes are degraded by hydrolytic enzymes active in an acidic environment of the lysosomal lumen.
An autophagic pathway consists of a series of signaling events activated in response to diverse stress and physiological conditions such as food deprivation,...
Delivery Pathways to the Lysosome01:36

Delivery Pathways to the Lysosome

Eukaryotic cells use different mechanisms to eliminate toxic waste obsolete and worn-out substances. Lysosomes play a pivotal role in this, and hence, these substances are carried to the lysosome from other parts of the cell and extracellular space through different pathways. The most elaborately studied pathways to the lysosome are the endocytic pathways.
Endocytosis
In endocytosis, the cell membrane takes up macromolecules and particles from the surrounding medium. Clathrin-mediated...
ER Retrieval Pathway01:45

ER Retrieval Pathway

In the secretory pathway, vesicles transport proteins from one cellular compartment to another in forward transport to deliver the protein to its correct location. Occasionally, misfolded proteins and incorrect proteins escape their original compartments, and a retrieval pathway is used to return the escaped proteins to their original compartment.
The ER uses many checkpoints to prevent the entry of incorrectly folded or a resident protein as cargo onto a transport vesicle. These mechanisms...
Autophagic Cell Death01:18

Autophagic Cell Death

Christian de Duve discovered “autophagy,” a process in which cellular components are engulfed by membrane-bound organelles called autophagosomes. The autophagosomes then fuse with lysosomes to digest the enclosed contents. Autophagy is generally activated in cells to prevent cell death. However, cell death is triggered when the damage is beyond repair.
Autophagy and Apoptosis
Autophagy can activate apoptosis. In normal conditions, the autophagy activating protein Beclin-1 and pro-apoptotic...
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,...
Export of Misfolded Proteins out of the ER01:32

Export of Misfolded Proteins out of the ER

After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...

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Related Experiment Video

Updated: Jun 26, 2026

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles
06:58

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles

Published on: October 18, 2024

ESCRT, autophagy, and frontotemporal dementia.

Jin-A Lee1, Fen-Biao Gao

  • 1Department of Neurology, University of California, San Francisco, CA, USA.

BMB Reports
|January 7, 2009
PubMed
Summary

Autophagy, a cellular cleanup process, is crucial for clearing toxic protein aggregates in neurodegenerative diseases. Mutations in CHMP2B disrupt this process, potentially causing frontotemporal dementia.

Area of Science:

  • Neurobiology
  • Cellular Biology
  • Genetics

Background:

  • Neurodegenerative diseases involve abnormal protein accumulation in neurons.
  • Autophagy is a key cellular pathway for degrading proteins and organelles.
  • The role of autophagy in neurodegeneration is not fully understood.

Purpose of the Study:

  • To investigate the role of the endosomal sorting complex required for transport (ESCRT) pathway in autophagy.
  • To explore the link between ESCRT components, protein aggregate clearance, and neurodegeneration.
  • To examine the impact of CHMP2B mutations on autophagosome-lysosome fusion.

Main Methods:

  • Studied the function of ESCRT subunits in the autophagy pathway.
  • Investigated the effect of reduced ESCRT activity on protein aggregate clearance.

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Ferritinophagy: Assessing the Selective Degradation of Iron by Autophagy in Human Fibroblasts
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Ferritinophagy: Assessing the Selective Degradation of Iron by Autophagy in Human Fibroblasts

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siRNA Electroporation to Modulate Autophagy in Herpes Simplex Virus Type 1-Infected Monocyte-Derived Dendritic Cells
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siRNA Electroporation to Modulate Autophagy in Herpes Simplex Virus Type 1-Infected Monocyte-Derived Dendritic Cells

Published on: October 28, 2019

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Last Updated: Jun 26, 2026

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles
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Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles

Published on: October 18, 2024

Ferritinophagy: Assessing the Selective Degradation of Iron by Autophagy in Human Fibroblasts
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Ferritinophagy: Assessing the Selective Degradation of Iron by Autophagy in Human Fibroblasts

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siRNA Electroporation to Modulate Autophagy in Herpes Simplex Virus Type 1-Infected Monocyte-Derived Dendritic Cells
09:10

siRNA Electroporation to Modulate Autophagy in Herpes Simplex Virus Type 1-Infected Monocyte-Derived Dendritic Cells

Published on: October 28, 2019

  • Analyzed the cellular consequences of CHMP2B mutations, focusing on autophagosome-lysosome fusion.

    • Main Results:

    • Reduced ESCRT subunit activity leads to autophagosome accumulation and impaired protein aggregate clearance.
    • Mutations in CHMP2B, an ESCRT-III subunit, are linked to frontotemporal dementia (FTD3).
    • Mutant CHMP2B disrupts autophagosome-lysosome fusion in cellular models.

    Conclusions:

    • The ESCRT pathway, particularly CHMP2B, plays a critical role in autophagy-mediated clearance of protein aggregates.
    • Dysfunctional autophagy due to CHMP2B mutations may underlie the pathogenesis of FTD3.
    • These findings suggest a novel mechanism for neurodegeneration involving impaired autophagosome-lysosome fusion.