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Related Concept Videos

Lysosomal Hydrolases01:22

Lysosomal Hydrolases

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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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Lysosomes01:31

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Lysosomes are membrane-enclosed spherical sacs derived from the Golgi apparatus. The most important function of the lysosome is degrading macromolecules and biological polymers that are released during membrane trafficking events such as the secretory, endocytic, autophagic, and phagocytic pathways. The degradation is carried out by several hydrolytic enzymes active in an acidic environment of the lysosomal lumen. These acid hydrolases are involved in cellular processes such as cell signaling,...
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Delivery Pathways to the Lysosome01:36

Delivery Pathways to the Lysosome

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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.
Endocytosis
In endocytosis, the cell membrane takes up macromolecules and particles from the surrounding medium. Clathrin-mediated...
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pH Regulation in Cells01:28

pH Regulation in Cells

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pH plays a critical role in maintaining normal cellular activities. It helps maintain the structure and function of various proteins, dictates the charge on cellular membranes, and is crucial for metabolic reactions inside the cell. Moreover, cells use the energy from the proton motive force to generate ATP.
Cytosolic pH
Under physiological conditions, the cytosolic pH is slightly more acidic than the extracellular pH. However, cells must prevent further acidification of their cytosol to...
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Liver Physiology01:30

Liver Physiology

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The liver, an essential organ in the human body, performs over 200 vital functions that can be broadly categorized into metabolic, hematological, endocrine regulation, and bile production.
Metabolic Regulation:
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Maturation of Endosomes01:28

Maturation of Endosomes

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The early endosome containing internalized molecules matures through transformations in its location, morphology, intraluminal pH, and membrane protein composition. Together, these changes result in a more acidic late endosome that contains multiple intraluminal vesicles; therefore, the late endosome is also called a multivesicular body (MVB).
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Related Experiment Video

Updated: Jan 11, 2026

Characterization of Neuronal Lysosome Interactome with Proximity Labeling Proteomics
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Characterization of Neuronal Lysosome Interactome with Proximity Labeling Proteomics

Published on: June 23, 2022

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Lysosomal physiology.

Haoxing Xu1, Dejian Ren

  • 1Department of Molecular, Cellular, and Developmental Biology, University of Michigan, Ann Arbor, Michigan 48109;

Annual Review of Physiology
|February 11, 2015
PubMed
Summary
This summary is machine-generated.

Lysosomes are vital acidic organelles degrading cellular waste via hydrolases. Dysfunctional lysosomal channels and transporters contribute to lysosomal storage diseases and neurodegeneration.

Keywords:
TFEBTPC1TPC2TRPML1lysosomal exocytosislysosomal storage diseasemTOR

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Area of Science:

  • Cell Biology
  • Molecular Biology
  • Biochemistry

Background:

  • Lysosomes are acidic cellular compartments containing over 60 hydrolases.
  • They are crucial for degrading extracellular and intracellular components via endocytosis and autophagy.
  • Lysosomes possess over 50 membrane proteins involved in nutrient sensing and regulating degradation processes.

Purpose of the Study:

  • To elucidate the role of lysosomal channels and transporters in cellular homeostasis.
  • To investigate the connection between lysosomal ion flux dysregulation and disease pathogenesis.
  • To highlight the importance of lysosomal function in metabolic and neurodegenerative disorders.

Main Methods:

  • Analysis of lysosomal hydrolase activity.
  • Investigation of endocytosis and autophagy pathways.
  • Characterization of lysosomal membrane proteins and their functions.
  • Study of ion flux across lysosomal membranes.
  • Examination of nutrient sensing mechanisms in lysosomes.

Main Results:

  • Lysosomes mediate degradation and transport of molecules via hydrolases, exporters, and trafficking.
  • Lysosomal nutrient sensing machinery influences organelle distribution, number, size, and activity.
  • Defects in lysosomal degradation, export, or trafficking lead to lysosomal dysfunction and storage diseases (LSDs).
  • Lysosomal channels and transporters regulate ion homeostasis, membrane potential, and catabolite export.
  • Dysregulation of these channels is implicated in LSDs and potentially other neurological and metabolic diseases.

Conclusions:

  • Lysosomal channels and transporters are critical for maintaining cellular ion balance and lysosomal function.
  • Their dysregulation is a key factor in the development of lysosomal storage diseases.
  • Understanding lysosomal transport mechanisms offers potential therapeutic targets for metabolic and neurodegenerative diseases.