Academic Journal
Structural Features of Heparan Sulfate from Multiple Osteochondromas and Chondrosarcomas
العنوان: | Structural Features of Heparan Sulfate from Multiple Osteochondromas and Chondrosarcomas |
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المؤلفون: | Noemi Veraldi, Alessandro Parra, Elena Urso, Cesare Cosentino, Manuela Locatelli, Serena Corsini, Elena Pedrini, Annamaria Naggi, Antonella Bisio, Luca Sangiorgi |
المصدر: | Molecules, Vol 23, Iss 12, p 3277 (2018) |
بيانات النشر: | MDPI AG, 2018. |
سنة النشر: | 2018 |
المجموعة: | LCC:Organic chemistry |
مصطلحات موضوعية: | heparan sulfate, multiple osteochondromas (MO), peripheral chondrosarcoma, human cartilage, NMR, HPLC–MS, EXT, Organic chemistry, QD241-441 |
الوصف: | Multiple osteochondromas (MO) is a hereditary disorder associated with benign cartilaginous tumors, known to be characterized by absence or highly reduced amount of heparan sulfate (HS) in the extracellular matrix of growth plate cartilage, which alters proper signaling networks leading to improper bone growth. Although recent studies demonstrated accumulation of HS in the cytoplasm of MO chondrocytes, nothing is known on the structural alterations which prevent HS from undergoing its physiologic pathway. In this work, osteochondroma (OC), peripheral chondrosarcoma, and healthy cartilaginous human samples were processed following a procedure previously set up to structurally characterize and compare HS from pathologic and physiologic conditions, and to examine the phenotypic differences that arise in the presence of either exostosin 1 or 2 (EXT1 or EXT2) mutations. Our data suggest that HS chains from OCs are prevalently below 10 kDa and slightly more sulfated than healthy ones, whereas HS chains from peripheral chondrosarcomas (PCSs) are mostly higher than 10 kDa and remarkably more sulfated than all the other samples. Although deeper investigation is still necessary, the approach here applied pointed out, for the first time, structural differences among OC, PCS, and healthy HS chains extracted from human cartilaginous excisions, and could help in understanding how the structural features of HS are modulated in the presence of pathological situations also involving different tissues. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 1420-3049 |
Relation: | https://www.mdpi.com/1420-3049/23/12/3277; https://doaj.org/toc/1420-3049 |
DOI: | 10.3390/molecules23123277 |
URL الوصول: | https://doaj.org/article/e9fd35e091064479ab6ab3046b405f00 |
رقم الانضمام: | edsdoj.9fd35e091064479ab6ab3046b405f00 |
قاعدة البيانات: | Directory of Open Access Journals |
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