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In Vitro Enzyme Measurement to Test Pharmacological Chaperone Responsiveness in Fabry and Pompe Disease
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Lysosomal positioning diseases: beyond substrate storage.

Gianluca Scerra1, Valeria De Pasquale2, Melania Scarcella1

  • 1Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Via Sergio Pansini 5, 80131 Naples, Italy.

Open Biology
|October 26, 2022
PubMed
Summary
This summary is machine-generated.

Lysosomal storage diseases (LSDs) result from enzyme deficiencies, causing substrate buildup and cellular damage. This review explores how lysosome positioning and movement link to LSDs progression.

Keywords:
lysosomal storage diseaseslysosomemembrane contact sitesmicrotubule trackspositioningtrafficking

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Area of Science:

  • Cell Biology
  • Genetics
  • Biochemistry

Background:

  • Lysosomal storage diseases (LSDs) are inherited disorders caused by deficiencies in lysosomal enzymes or proteins.
  • Substrate accumulation in lysosomes impairs cellular homeostasis and damages organs.
  • Lysosomes have roles beyond waste clearance, impacting various physiological and pathological processes.

Purpose of the Study:

  • To review recent advances in understanding the link between lysosome positioning, trafficking, and LSDs.
  • To highlight the molecular mechanisms involved in LSD pathogenesis beyond substrate accumulation.

Main Methods:

  • Literature review of recent studies on lysosomal biology and LSDs.
  • Analysis of cellular and molecular mechanisms connecting lysosome dynamics to disease.

Main Results:

  • Lysosomal substrate accumulation triggers a cascade of detrimental cellular events.
  • Impaired lysosome positioning and trafficking contribute significantly to LSD pathology.
  • Lysosome dysfunction affects cellular processes like metabolism, signaling, and organelle function.

Conclusions:

  • Lysosome positioning and trafficking are critical factors in LSD pathogenesis.
  • Further research into these mechanisms may reveal novel therapeutic targets for LSDs.