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Related Concept Videos

Lysosomal Hydrolases01:22

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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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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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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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Nuclear protein sorting regulates nucleus composition and gene expression, crucial for determining the fate of a eukaryotic cell. Hence, the entry and exit of molecules across the nuclear envelope is a tightly controlled process. Nuclear protein sorting can be inhibited by one of the following ways: 1) masking cargo signal sequences, 2) modifying the nuclear receptor's affinity for cargo, 3) controlling the nuclear pore size, 4) retaining the cargo during its transit to the cytosol or the...
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The ER, Golgi apparatus, endosomes, and lysosomes work in tandem to modify, sort, and package proteins and lipids. An integrated membrane trafficking network facilitates the back and forth shuttling of molecules within different organelles in the same cell or across the cell membrane.
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The recycling endosome, also known as the endosomal recycling compartment (ERC), is a part of the slow-recycling process of the endocytic pathway. Molecules internalized through receptor-mediated endocytosis are either degraded in the lysosomes or are recycled to the plasma membrane through the fast- or slow-recycling route.
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Characterization of Neuronal Lysosome Interactome with Proximity Labeling Proteomics
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Lysosomal Trafficking Regulator (LYST).

Xiaojie Ji1,2, Bo Chang3, Jürgen K Naggert4

  • 1The Jackson Laboratory, 04609, Bar Harbor, ME, USA. xiaojie.ji@jax.org.

Advances in Experimental Medicine and Biology
|October 3, 2015
PubMed
Summary

Disrupting the lysosomal trafficking regulator (LYST) causes Chediak-Higashi syndrome (CHS), leading to enlarged organelles and severe health issues. Understanding LYST function may reveal new therapeutic targets for CHS and related disorders.

Keywords:
Chediak-Higashi syndromeLysosomal trafficking regulator (LYST)LysosomeLysosome-related organellesMelanosomeRetinal Pigment Epithelium (RPE)Vesicle trafficking

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Area of Science:

  • Cell Biology
  • Genetics
  • Immunology

Background:

  • Lysosome-related organelle (LRO) size and vesicle trafficking are vital for cellular function.
  • Disruption of the lysosomal trafficking regulator (LYST) causes Chediak-Higashi syndrome (CHS), a rare genetic disorder.
  • CHS is characterized by enlarged LROs, oculocutaneous albinism, immunodeficiency, and neurologic dysfunction.

Purpose of the Study:

  • To elucidate the molecular function of LYST.
  • To identify LYST interacting partners.
  • To explore therapeutic targets for CHS and related diseases.

Main Methods:

  • The abstract does not specify the methods used.
  • Further research is needed to detail the experimental approaches.

Main Results:

  • LYST disruption leads to enlarged LROs, including lysosomes, melanosomes, and cytolytic granules.
  • Ocular pathology in CHS includes enlarged melanosomes in the retinal pigment epithelium (RPE), causing visual impairment.
  • CHS patients exhibit oculocutaneous albinism, bleeding issues, and severe immunodeficiency.

Conclusions:

  • Understanding LYST's molecular function and interactions is crucial for developing CHS therapies.
  • LYST plays a key role in regulating organelle size and vesicle trafficking.
  • Therapeutic strategies targeting LYST may benefit patients with CHS and other conditions like asthma and Leishmania infections.