Mikami Tadahisa, Kitagawa Hiroshi
Department of Biochemistry, Kobe Pharmaceutical University, Higashinada-ku, Kobe, Japan.
Biochim Biophys Acta. 2013 Oct;1830(10):4719-33. doi: 10.1016/j.bbagen.2013.06.006. Epub 2013 Jun 14.
Chondroitin sulfate proteoglycans (CSPGs) are principal pericellular and extracellular components that form regulatory milieu involving numerous biological and pathophysiological phenomena. Diverse functions of CSPGs can be mainly attributed to structural variability of their polysaccharide moieties, chondroitin sulfate glycosaminoglycans (CS-GAG). Comprehensive understanding of the regulatory mechanisms for CS biosynthesis and its catabolic processes is required in order to understand those functions.
Here, we focus on recent advances in the study of enzymatic regulatory pathways for CS biosynthesis including successive modification/degradation, distinct CS functions, and disease phenotypes that have been revealed by perturbation of the respective enzymes in vitro and in vivo.
Fine-tuned machineries for CS production/degradation are crucial for the functional expression of CS chains in developmental and pathophysiological processes.
Control of enzymes responsible for CS biosynthesis/catabolism is a potential target for therapeutic intervention for the CS-associated disorders.
硫酸软骨素蛋白聚糖（CSPGs）是主要的细胞周和细胞外成分，形成涉及众多生物学和病理生理学现象的调节环境。CSPGs的多种功能主要归因于其多糖部分硫酸软骨素糖胺聚糖（CS-GAG）的结构变异性。为了理解这些功能，需要全面了解CS生物合成及其分解代谢过程的调节机制。
在此，我们重点关注CS生物合成酶促调节途径的研究进展，包括连续修饰/降解、不同的CS功能以及通过体外和体内干扰各自酶所揭示的疾病表型。
CS产生/降解的精细调节机制对于CS链在发育和病理生理过程中的功能表达至关重要。
控制负责CS生物合成/分解代谢的酶是CS相关疾病治疗干预的潜在靶点。
