Regular paperProteoglycan sulfation in cartilage and cell cultures from patients with sulfate transporter chondrodysplasias: Relationship to clinical severity and indications on the role of intracellular sulfate production
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Biology of Proteoglycans and Associated Glycosaminoglycans
2021, Comprehensive Glycoscience: Second EditionImprovement of the skeletal phenotype in a mouse model of diastrophic dysplasia after postnatal treatment with N-acetylcysteine
2021, Biochemical PharmacologyCitation Excerpt :Nowadays patients are treated by corrective orthopaedic surgery and physiotherapy, but no pharmacological treatment is available. Since a correlation between the severity of the clinical phenotype and cartilage PG undersulfation was demonstrated in SLC26A2 chondrodysplasias [4], a pharmacological strategy targeted at increasing cartilage PG sulfation would likely result in an improvement of the skeletal phenotype. In chondrocytes, beyond extracellular sulfate uptake, a small amount of sulfate may come from the catabolism of sulfur-containing amino acids such as methionine and cysteine.
Calcium activated nucleotidase 1 (CANT1) is critical for glycosaminoglycan biosynthesis in cartilage and endochondral ossification
2019, Matrix BiologyCitation Excerpt :The released chondrocytes were plated and cultured in DMEM with 10% foetal calf serum (FCS) (EuroClone) at 37 °C in a humidified atmosphere containing 5% CO2. Chondrocyte cultures were preincubated with or without 1 mM p-nitrophenyl β-d-xylopyranoside (Sigma-Aldrich) in minimal essential medium (MEM) (Sigma-Aldrich) containing 250 μM cold Na2SO4 without FCS at 37 °C in 5% CO2 for 2 h. Cells were then labelled with 50 μCi/ml Na2[35SO4] (38.8–59.2 TBq/mmol, PerkinElmer) in the same medium for 24 h as described previously [40]. At the end of the labelling period, an equal volume of 100 mM sodium acetate buffer, pH 5.8, containing 8 M urea, 4% Triton X-100, 20 mM EDTA, 20 mM N-ethylmalemide (NEM), 0.1 M 6-aminocaproic acid and 1 mM phenylmethylsulfonyl fluoride (PMSF) was added to the medium.
Suppressing UPR-dependent overactivation of FGFR3 signaling ameliorates SLC26A2-deficient chondrodysplasias
2019, EBioMedicineCitation Excerpt :Safranin O, a cationic dye showing affinity to polyanionic sulfated molecules, was used to reveal the sulfation degree of the ECM. Consistent with previous studies on human ACG1B and AO2 [48], the cartilage matrix was less intensely stained by Safranin O in slc26a2−/− growth plates than slc26a2+/− controls, which indicated matrix undersulfation (Fig. 1I). The morphology of slc26a2−/− growth plates was severely disrupted, showing shortened length, disorganized chondrocyte alignment and decreased cellularity (Fig. 1I-K).