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Related Concept Videos

Protein Import into the Peroxisomes01:27

Protein Import into the Peroxisomes

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Cells contain membrane-bound organelles called peroxisomes that oxidize organic molecules by transferring hydrogen atoms to oxygen, producing hydrogen peroxide. Peroxisomes enzymatically convert the released hydrogen peroxide into water and oxygen.
Peroxisomal Protein Import:
Peroxisomes lack the genetic machinery required to code for their own proteins. Hence, most peroxisomal membrane, lumenal and transmembrane proteins are synthesized in the cytoplasm or ER and transported to the peroxisome...
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Peroxisomes01:24

Peroxisomes

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Peroxisomes are specialized organelles present in fungi, plant, and animal cells. It can vary in number, size, morphology, and activity depending on the type of tissue and the nutritional state of the cell. For example, cells with active lipid metabolism, such as adipocytes, neurons, and hepatocytes, have more peroxisomes than other cells in the body. Besides their primary role in breaking down complex organic molecules, peroxisomes can also synthesize specific macromolecules and participate in...
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Autophagy01:27

Autophagy

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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.
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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.
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Export of Misfolded Proteins out of the ER01:32

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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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Monitoring Stub1-Mediated Pexophagy
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Peroxisomal protein PEX13 functions in selective autophagy.

Ming Y Lee1, Rhea Sumpter1, Zhongju Zou1,2

  • 1Center for Autophagy Research, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.

EMBO Reports
|November 10, 2016
PubMed
Summary

PEX13 protein is crucial for selective autophagy, including the removal of damaged mitochondria. Disease-associated PEX13 mutations impair this mitophagy function, potentially contributing to Zellweger spectrum disorders.

Keywords:
PEX13Zellweger syndromeautophagymitophagyvirophagy

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Area of Science:

  • Cell Biology
  • Molecular Biology
  • Genetics

Background:

  • Peroxisome biogenesis disorders (PBDs), such as Zellweger syndrome spectrum (ZSS) disorders, are linked to mutations in peroxin (PEX) genes.
  • ZSS disorders cause severe neurological, hepatic, and renal abnormalities, often leading to neonatal death, with impaired peroxisome function considered the primary cause.
  • The precise mechanisms underlying ZSS pathogenesis remain incompletely understood.

Purpose of the Study:

  • To investigate the role of PEX13 beyond peroxisome import.
  • To determine if PEX13 is involved in selective autophagy pathways.
  • To assess the impact of disease-associated PEX13 mutations on its cellular functions.

Main Methods:

  • Utilized cell-based assays to examine the function of PEX13 in selective autophagy.
  • Investigated the role of PEX13 in virophagy (viral clearance) and mitophagy (damaged mitochondria removal).
  • Analyzed specific PEX13 mutants (I326T and W313G) for defects in mitophagy.

Main Results:

  • Demonstrated that PEX13 is essential for selective autophagy, specifically for virophagy and mitophagy.
  • Identified that disease-associated PEX13 mutants I326T and W313G exhibit impaired mitophagy.
  • Showed that PEX13's mitophagy function is shared with PEX3 but distinct from PEX14 and PEX19, which are involved in general autophagy.

Conclusions:

  • PEX13 plays a critical role in selective autophagy processes.
  • Dysfunctional PEX13-mediated mitophagy may be a contributing factor to the pathogenesis of Zellweger spectrum disorders.
  • This study expands the known functions of PEX13 and offers new insights into PBDs.