Beck M, Lingnau K, Spranger J
Department of Pediatrics, University of Mainz, Federal Republic of Germany.
Bone. 1988;9(6):367-70. doi: 10.1016/8756-3282(88)90118-4.
In an attempt to elucidate the biochemical defect in pseudoachondroplasia, proteoglycan metabolism was investigated in cartilage from a patient with the dominant form of this condition. Iliac-crest cartilage was radioactively labeled with 35S-sulfate and the newly synthesized proteoglycans examined for their hydrodynamic size and glycosaminoglycan composition. The banding pattern of the purified proteoglycans was analyzed by gel-electrophoresis using large pore polyacrylamide-agarose. We found a normal chain-length of glycosaminoglycans and a normal ratio of chondroitin-6-sulfate to chondroitin-4-sulfate. The proteoglycans were not enriched in keratan sulfate. Gel electrophoretic analysis of the proteoglycans disclosed a banding pattern comparable to that of two normal controls. In contrast to the findings of other authors no differences between the proteoglycans of pseudoachondroplastic and normal cartilage were detected.
为了阐明假性软骨发育不全的生化缺陷，对一名患有这种显性疾病的患者的软骨中的蛋白聚糖代谢进行了研究。用35S-硫酸盐对髂嵴软骨进行放射性标记，并检查新合成的蛋白聚糖的流体力学大小和糖胺聚糖组成。使用大孔聚丙烯酰胺-琼脂糖通过凝胶电泳分析纯化的蛋白聚糖的条带模式。我们发现糖胺聚糖的链长正常，硫酸软骨素-6与硫酸软骨素-4的比例正常。蛋白聚糖中硫酸角质素没有富集。对蛋白聚糖的凝胶电泳分析显示出与两个正常对照相当的条带模式。与其他作者的发现相反，未检测到假性软骨发育不全软骨和正常软骨的蛋白聚糖之间存在差异。
