Relationship of Lysosomal Storage Diseases (LSD) with Autophagy

Lysosomes are organelles that degrade damaged components or structures that have completed their functions and have roles in the last step of the autophagy pathway. Damage of the autophagy-lysosome pathway can cause vital problems for the cell. Lysosomal storage diseases (LSD) are one of the most co...

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Main Authors: Seda Keskin, İsmail Mert Alkaç, Burcu Çerçi
Format: Article
Language:English
Published: Van Yuzuncu Yil University, School of Medicine 2022-10-01
Series:Van Tıp Dergisi
Subjects:
Online Access:https://jag.journalagent.com/z4/download_fulltext.asp?pdir=vtd&un=VTD-95776
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author Seda Keskin
İsmail Mert Alkaç
Burcu Çerçi
author_facet Seda Keskin
İsmail Mert Alkaç
Burcu Çerçi
author_sort Seda Keskin
collection DOAJ
description Lysosomes are organelles that degrade damaged components or structures that have completed their functions and have roles in the last step of the autophagy pathway. Damage of the autophagy-lysosome pathway can cause vital problems for the cell. Lysosomal storage diseases (LSD) are one of the most common human genetic disorder caused by gene mutations. Up to now, more than 70 LSD were identified. LSD is mainly caused by functional disorders of enzymes in lysosomes or proteins associated with lysosomes. These disorders lead to the accumulation of undigested macromolecules in cells LSD can cause many systemic damages mainly in the nervous system, skeletal system, and reticuloendothelial system especially at early stages of the disease. Modulation and reactivation of autophagy is accepted as a new therapeutic approach for LSD. In this review, the general mechanisms of the relationship between lysosomal storage diseases and autophagy were evaluated together with the treatment approaches.
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spelling doaj.art-d15d22d997204b0c805889e908909de62023-12-03T08:29:23ZengVan Yuzuncu Yil University, School of MedicineVan Tıp Dergisi2587-03512022-10-0129444545310.5505/vtd.2022.95776VTD-95776Relationship of Lysosomal Storage Diseases (LSD) with AutophagySeda Keskin0İsmail Mert Alkaç1Burcu Çerçi2Van Yüzüncü Yıl University, Faculty of Medicine Department of Medical Histology and Embryology, VanDumlupınar University, Faculty of Engineering, Department of Materials Science Engineering, Kütahyaİzmir Katip Çelebi University, Faculty of Medicine, Department of Medical Biology and Genetics, İzmirLysosomes are organelles that degrade damaged components or structures that have completed their functions and have roles in the last step of the autophagy pathway. Damage of the autophagy-lysosome pathway can cause vital problems for the cell. Lysosomal storage diseases (LSD) are one of the most common human genetic disorder caused by gene mutations. Up to now, more than 70 LSD were identified. LSD is mainly caused by functional disorders of enzymes in lysosomes or proteins associated with lysosomes. These disorders lead to the accumulation of undigested macromolecules in cells LSD can cause many systemic damages mainly in the nervous system, skeletal system, and reticuloendothelial system especially at early stages of the disease. Modulation and reactivation of autophagy is accepted as a new therapeutic approach for LSD. In this review, the general mechanisms of the relationship between lysosomal storage diseases and autophagy were evaluated together with the treatment approaches.https://jag.journalagent.com/z4/download_fulltext.asp?pdir=vtd&un=VTD-95776autophagylysosomal storage diseaseendosomeprotein function loss
spellingShingle Seda Keskin
İsmail Mert Alkaç
Burcu Çerçi
Relationship of Lysosomal Storage Diseases (LSD) with Autophagy
Van Tıp Dergisi
autophagy
lysosomal storage disease
endosome
protein function loss
title Relationship of Lysosomal Storage Diseases (LSD) with Autophagy
title_full Relationship of Lysosomal Storage Diseases (LSD) with Autophagy
title_fullStr Relationship of Lysosomal Storage Diseases (LSD) with Autophagy
title_full_unstemmed Relationship of Lysosomal Storage Diseases (LSD) with Autophagy
title_short Relationship of Lysosomal Storage Diseases (LSD) with Autophagy
title_sort relationship of lysosomal storage diseases lsd with autophagy
topic autophagy
lysosomal storage disease
endosome
protein function loss
url https://jag.journalagent.com/z4/download_fulltext.asp?pdir=vtd&un=VTD-95776
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