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遗传性软骨发育不良小鼠软骨的扫描电子显微镜观察

Scanning electron microscopy of cartilage in mice with hereditary chondrodysplasia.

作者信息

Seegmiller R E, Monson C B

出版信息

Scan Electron Microsc. 1982(Pt 3):1259-67.

PMID:6820573
Abstract

Mice born with hereditary, recessive chondrodysplasia (cho/cho) are dwarfed because the cartilage model upon which the endochondral osseous skeleton develops is defective. The mutant's cartilage matrix lacks cohesiveness which apparently contributes to the absence of columnar alignment of proliferating epiphyseal chondrocytes in developing tubular (long) bones. The present communication reviews our current understanding of skeletal dysplasia as it relates to defective chondrogenesis, and presents observations made with the scanning electron microscope of cellular disarray and nonuniform size and distribution of collagen fibrils which confirm the existence of a matrix defect. Autoradiographic experiments on tibial cartilage, similar to those performed on sternal cartilage, confirm the normal pattern of sulfate labeling by mutant epiphyses.

摘要

患有遗传性隐性软骨发育不良(cho/cho)的小鼠体型矮小,因为软骨内成骨骨骼发育所依赖的软骨模型存在缺陷。突变体的软骨基质缺乏黏附性,这显然导致了发育中的管状(长)骨中增殖性骨骺软骨细胞缺乏柱状排列。本通讯综述了我们目前对与软骨生成缺陷相关的骨骼发育不良的理解,并展示了扫描电子显微镜观察到的细胞紊乱以及胶原纤维大小和分布不均的现象,这些现象证实了基质缺陷的存在。对胫骨软骨进行的放射自显影实验,与对胸骨软骨进行的实验类似,证实了突变体骨骺硫酸盐标记的正常模式。

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