[MOLECULAR GENETIC ASPECTS OF THE DEVELOPMENT OF THE MUCOPOLYSACCHARIDOSES (REVIEW)].

The review highlights the current knowledge about the potential role of glycosaminoglycans in the induction of inflammation and development of damage of the functional systems of the organs by mucopolysaccharides (MPS). Undegraded glycosaminoglycans are stimulants of secondary events in the form of complex pathogenetic cascades: accumulation of secondary substrates unrelated to the defective enzyme, abnormal composition of the membranes, disorders of intracellular vesicular transport, impairment of autophagy, change of intracellular signaling (aberrant activation of signaling pathways), abnormalities of calcium homeostasis, oxidative stress. Understanding of the cellular processes underlying the pathophysiology of MPS helps to address the limitations of the existing therapies and to identify new therapeutic targets, which potentially form additional and effective ways of the therapy of the patients with MPS.