Reduction of lysosome abundance and GAG accumulation after odiparcil treatment in MPS I and MPS VI models
Deficiencies of lysosomal enzymes responsible for the degradation of glycosaminoglycans (GAG) cause pathologies commonly known as the mucopolysaccharidoses (MPS). Each type of MPS is caused by a deficiency in a specific GAG-degrading enzyme and is characterized by an accumulation of disease-specific...
Main Authors: | , , , , , , |
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格式: | Article |
語言: | English |
出版: |
Elsevier
2023-12-01
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叢編: | Molecular Genetics and Metabolism Reports |
主題: | |
在線閱讀: | http://www.sciencedirect.com/science/article/pii/S2214426923000575 |