title: Extracellular matrix components: an intricate network of possible biomarkers for lysosomal storage disorders?
abstract: Biomarkers are extremely important in the case of multisystemic diseases, such as lysosomal storage disorders (LSDs), which are often difficult to assess in clinical practice. Several studies demonstrated significant alterations in the expression of extracellular matrix (ECM) components in LSD patients, raising important questions in relation to their possible involvement in disease pathogenesis and providing evidence for their possible utility as disease biomarkers. This article provides an overview of the possible pathogenic correlations between LSDs and ECM. Data regarding the expression of these molecules are discussed. Finally, the possible implication of ECM components as therapeutic targets in this group of diseases along with the impact of the differential expression of these components in current LSD treatment will be critically addressed.
authors: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration General Hospital, Thessaloniki, Greece.
authors: Spyros P
authors: SP
authors: Batzios
authors: Dimitrios I
authors: DI
authors: Zafeiriou
authors: Eleni
authors: E
authors: Papakonstantinou
mesh_terms: D000818:Animals; D015415:Biomarkers / Q000378:metabolism; D005109:Extracellular Matrix / Q000187:drug effects / Q000378:metabolism*; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000378:metabolism*; D020869:Gene Expression Profiling; D006025:Glycosaminoglycans / Q000378:metabolism*; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism*; D015398:Signal Transduction / Q000187:drug effects / Q000235:genetics
chemical_list: D015415:Biomarkers; D016326:Extracellular Matrix Proteins; D006025:Glycosaminoglycans