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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,...

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Related Experiment Video

Updated: Mar 23, 2026

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

Published on: October 21, 2014

Endothelial cell dysfunction in globoid cell leukodystrophy.

Mirella Belleri¹, Marco Presta²

¹Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

Journal of Neuroscience Research

|April 3, 2016

Summary

Globoid cell leukodystrophy (GLD) involves brain microvascular damage, potentially due to the toxic metabolite psychosine. Endothelial cell dysfunction may be a key factor in GLD pathogenesis and offers therapeutic targets.

Keywords:

Krabbe disease angiogenesis central nervous system endothelial cells psychosine twitcher mouse

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Area of Science:

Neuroscience
Vascular Biology
Lysosomal Storage Diseases

Background:

Angiogenesis is crucial for brain function and pathology.
Microvascular changes are evident in neurodegenerative diseases, including genetic leukodystrophies.
Globoid cell leukodystrophy (GLD) is a lysosomal storage disease linked to beta-galactosylceramidase (GALC) deficiency, causing toxic psychosine accumulation.

Purpose of the Study:

To investigate microvascular and endothelial cell alterations in Globoid cell leukodystrophy (GLD).
To explore the role of psychosine and GALC deficiency in endothelial cell dysfunction.
To identify potential novel pathogenic mechanisms and therapeutic strategies for GLD.

Main Methods:

Examined structural and functional changes in brain microvasculature of GLD patients and twitcher mice.
Assessed endothelial cell permeability and response to proangiogenic stimuli in twitcher mice.
Investigated psychosine's angiostatic effects and GALC downregulation impacts using in vivo (zebrafish) and in vitro (human endothelial cells) models.

Main Results:

GLD is associated with structural and functional alterations in brain microvascular endothelium.
Endothelium in twitcher mice exhibits increased permeability and impaired response to proangiogenic factors.
Psychosine may exert angiostatic effects, and GALC downregulation can cause psychosine-independent vascular defects.

Conclusions:

Endothelial cell dysfunction represents a potential novel pathogenic mechanism in GLD and other leukodystrophies.
Understanding these microvascular alterations may lead to new therapeutic approaches for GLD.
Further research into the molecular mechanisms of these vascular defects is warranted.