Experimental Models of CNS Deficits in Lysosomal Storage Diseases

Author:

Lebedev A. S.123,Kotova M. M.2,Kolesnikova T. O.2,Galstyan D. S.134,Kalueff A. V.12345

Affiliation:

1. World-class Scientific Research Center “Center of Personalized Medicine”, Almazov National Medical Research Center, Ministry of Healthcare of Russian Federation

2. Neurobiology Department, Research Center for Genetics and Life Sciences, Sirius University of Science and Technology

3. Institute of Translational Biomedicine, St. Petersburg State University

4. Granov Russian Scientific Center for Radiology and Surgical Technologies, Ministry of Healthcare of Russian Federation

5. Ural Federal University

Abstract

Lysosomal storage diseases are characterized by enzyme deficiency in the lysosomal appar-atus of the cell, triggering a pathological accumulation of undigested cellular material (proteins, lipids or carbohydrates) and tissue damage. Clinically and etiologically dive-rse, this group includes over 70 presently recognized hereditary conditions with no known effective therapy. Thus, the search for therapeutic strategies directed at these disorders represents an urgent unmet biomedical task, also necessitating the use of appropriate and valid experimental (animal) models. Here, we discuss the existing models of lysosomal storage diseases and the applicability of rodent and zebrafish as model organisms for probing these diseases.

Publisher

The Russian Academy of Sciences

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Translational Cross-Taxon and Cross-Domain CNS Studies;Российский физиологический журнал им  И  М  Сеченова;2023-11-01

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