title: Changes in the metabolism of chondroitin sulfate glycosaminoglycans in articular cartilage from patients with Kashin-Beck disease.
abstract: OBJECTIVE
  To identify changes in the expression patterns of enzymes involved in chondroitin sulfate (CS) glycosaminoglycan (GAG) metabolism in articular cartilage proteoglycan (PG) isolated from adolescent patients with Kashin-Beck disease (KBD).
  
  
  METHODS
  Samples of articular cartilage were divided into two groups: Control samples (from five normal children), and KBD samples (from five KBD children) aged 3-12 years old. The morphology and pathology of hand joint cartilage were examined by histochemical staining. The localization and expression patterns of enzymes involved in CS GAG metabolism (i.e., PAPS synthetase 2 (PAPSS2), PAPS transporter 1 (PAPST1), Carbohydrate (N-acetylgalactosamine 4-sulfate 6-O) sulfotransferases 15 (CHST15), Arylsulfatase B (ARSB) and N-acetylgalactosamine-6-sulfate sulfatase (GALNS)) were performed using immuno-histochemical analyses. Positive immunostaining in articular cartilage was semi-quantified.
  
  
  RESULTS
  Reduced aggrecan staining was observed in KBD samples compared with the control samples. The percentages of positive staining for the anabolic enzymes PAPSS2, PAPST1 and CHST15 in the upper and middle zones of KBD samples were significantly lower than that found in the Controls. In contrast, the percentages of positive staining in KBD samples for the catabolic enzymes ARSB and GALNS were significantly higher than the control samples. However, the staining for all of these GAG metabolism enzymes were hardly observed in the deep zones of KBD cartilage, suggesting that significant cell death and necrosis had occurred in this region.
  
  
  CONCLUSIONS
  Our results indicate that alterations of enzymes involved in articular cartilage CS GAG metabolism on PGs in the articular cartilage play an important role in the onset and pathogenesis of KBD in adolescent children.
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: luomingxiuwater@126.com.
authors.forename: M
authors.initials: M
authors.lastname: Luo
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: jixiang46@163.com.
authors.forename: J
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Anesthesiology, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an 710004, Shaanxi, China. Electronic address: foxlsy@163.com.
authors.forename: S
authors.initials: S
authors.lastname: Li
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: sunhongzhi@mail.xjtu.edu.cn.
authors.forename: H
authors.initials: H
authors.lastname: Sun
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: zhangmen509507@21cn.com.
authors.forename: Z
authors.initials: Z
authors.lastname: Zhang
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: fuqiang0303@gmail.com.
authors.forename: Q
authors.initials: Q
authors.lastname: Fu
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: kahafa@126.com.
authors.forename: J
authors.initials: J
authors.lastname: Li
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: 378762018@qq.com.
authors.forename: J
authors.initials: J
authors.lastname: Wang
authors.affiliation: Connective Tissue Biology Laboratories, Division of Pathophysiology and Repair, School of Biosciences, Cardiff University, Cardiff CF10 3AX, Wales, UK. Electronic address: hughesce1@cardiff.ac.uk.
authors.forename: C E
authors.initials: CE
authors.lastname: Hughes
authors.affiliation: Connective Tissue Biology Laboratories, Division of Pathophysiology and Repair, School of Biosciences, Cardiff University, Cardiff CF10 3AX, Wales, UK. Electronic address: caterson@cardiff.ac.uk.
authors.forename: B
authors.initials: B
authors.lastname: Caterson
authors.affiliation: Institute of Endemic Diseases, Xi'an Jiaotong University College of Medicine, Xi'an 710061, Shaanxi, China; Key Laboratory of Environment and Genes Related to Diseases (Xi'an Jiaotong University), Ministry of Education, Xi'an 710061, Shaanxi, China. Electronic address: caojl@mail.xjtu.edu.cn.
authors.forename: J
authors.initials: J
authors.lastname: Cao
mesh_terms: D053674:Aggrecans / Q000378:metabolism; D002358:Cartilage, Articular / Q000378:metabolism* / Q000473:pathology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002809:Chondroitin Sulfates / Q000378:metabolism*; D005260:Female; D006801:Humans; D057767:Kashin-Beck Disease / Q000209:etiology / Q000378:metabolism*; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D026901:Membrane Transport Proteins / Q000378:metabolism; D009097:Multienzyme Complexes / Q000378:metabolism; D011509:Proteoglycans / Q000378:metabolism; D013430:Sulfate Adenylyltransferase / Q000378:metabolism; D000076988:Sulfate Transporters; D015238:Sulfotransferases / Q000378:metabolism
chemical_list: D053674:Aggrecans; C411874:CHST15 protein, human; D008562:Membrane Glycoproteins; D026901:Membrane Transport Proteins; D009097:Multienzyme Complexes; D011509:Proteoglycans; C476248:SLC35B2 protein, human; D000076988:Sulfate Transporters; C038142:chondroitin sulfate glycosaminoglycan; D002809:Chondroitin Sulfates; C022322:PAPS synthetase; D013430:Sulfate Adenylyltransferase; D015238:Sulfotransferases
keywords: Aggrecan; Cartilage; Glycosaminoglycan sulfation; Kashin–Beck disease