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Lysosomal Hydrolases

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,...

Lysosomes

Lysosomes

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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Related Experiment Video

Updated: May 8, 2026

Mouse Model of Metabolic Dysfunction-Associated Steatotic Liver Disease with Fibrosis

Mouse Model of Metabolic Dysfunction-Associated Steatotic Liver Disease with Fibrosis

Published on: July 18, 2025

Animal models for lysosomal storage disorders.

G M Pastores¹, P A Torres, B-J Zeng

¹Neurogenetics, Department of Neurology, New York University School of Medicine, New York, NY 10016, USA. Gregory.Pastores@nyumc.org

Biochemistry. Biokhimiia

|September 10, 2013

Summary

This summary is machine-generated.

Animal models offer valuable insights into lysosomal storage disorders (LSDs), aiding therapeutic development. However, larger models may better replicate human brain pathology for enhanced treatment strategies.

Related Experiment Videos

Last Updated: May 8, 2026

Mouse Model of Metabolic Dysfunction-Associated Steatotic Liver Disease with Fibrosis

Mouse Model of Metabolic Dysfunction-Associated Steatotic Liver Disease with Fibrosis

Published on: July 18, 2025

Area of Science:

Biochemistry
Genetics
Pharmacology

Background:

Lysosomal storage disorders (LSDs) are inherited diseases caused by deficiencies in lysosomal hydrolases.
These deficiencies lead to the accumulation of macromolecules like glycoproteins and glycolipids.
Animal models have been crucial for understanding LSD mechanisms and testing therapies.

Purpose of the Study:

To review lessons learned from animal models in LSD research.
To highlight the utility of animal models in elucidating disease mechanisms and therapeutic concepts.
To discuss the limitations of current models and the need for larger animal models.

Main Methods:

Review of scientific literature published between 2011-2012.
Analysis of insights gained from various animal models of LSDs.
Comparison of disease manifestation in animal models versus human patients.

Main Results:

Animal models have advanced understanding of molecular changes preceding LSD symptoms.
Therapies like enzyme replacement and substrate reduction have been developed based on animal studies.
Certain mouse models exhibit biochemical defects but not the full disease course seen in humans.

Conclusions:

Animal models are indispensable for LSD research and therapeutic proof-of-concept.
Larger spontaneous animal models may offer better human disease mimicry, particularly for brain pathology.
Continued research using appropriate animal models is essential for developing effective LSD treatments.