短尾小鼠软骨生长板的缺陷。
Defects in the cartilaginous growth plates of brachymorphic mice.
作者信息
Orkin R W, Williams B R, Cranley R E, Poppke D C, Brown K S
出版信息
J Cell Biol. 1977 May;73(2):287-99. doi: 10.1083/jcb.73.2.287.
Abstract
Homozygous brachymorphic (bm/bm) mice are characterized by disproportionately short stature. Newborn bm/bm epiphyseal cartilages are shorter than normal although the cells in the different zones of growth are relatively well organized. The extracellular matrix reacts poorly with stains specific for sulfated glycosaminoglycans. The ultrastructural appearance of the cartilage matrix indicates normal collagen fibrils; however, proteoglycan aggregate granules are smaller than normal and are present in reduced numbers, particularly in the columnar and hypertrophic zones of the growth plate. In addition, a prominent network of fine filaments, which are extractable in 4 M guanidine hydrochloride, are present in the bm/bm cartilage matrix. These findings suggest that a defect affecting the proteoglycan component of cartilage occurs in bm/bm mice.
摘要
纯合短形（bm/bm）小鼠的特征是身材极短。新生bm/bm骨骺软骨比正常的短，尽管生长不同区域的细胞组织相对良好。细胞外基质与硫酸化糖胺聚糖特异性染色反应不佳。软骨基质的超微结构显示胶原原纤维正常；然而，蛋白聚糖聚集颗粒比正常的小且数量减少，特别是在生长板的柱状和肥大区域。此外，bm/bm软骨基质中存在一个可在4M盐酸胍中提取的细丝状突出网络。这些发现表明，bm/bm小鼠的软骨蛋白聚糖成分存在缺陷。
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