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骨硬化大鼠突变体“无齿”中缺乏佝偻病的证据。

Lack of evidence for rickets in the osteopetrotic rat mutation, toothless.

作者信息

Seifert M F

机构信息

Department of Anatomy, Indiana University School of Medicine, Indianapolis.

出版信息

J Bone Miner Res. 1994 Nov;9(11):1813-21. doi: 10.1002/jbmr.5650091119.

Abstract

A common, but paradoxic, feature among osteopetrotic human infants is the presence of rickets. This disorder of mineralization is manifested radiographically and histologically by increased growth plate cartilage and hypertrophic cell zone thickness and excess metaphyseal osteoid and biochemically by decreased serum calcium and phosphorus concentrations. Rickets has also been reported in two osteopetrotic animal mutations, the osteosclerotic (oc) mouse and the toothless (tl) rat. Although the phenotypic expression of the rachitic lesion in the oc mouse closely resembles that in affected humans, the results of the present study show that the lesion in the tl rat does not. Compared with normal littermates, histologic and morphometric analyses of tibial growth plate cartilage in tl rats up to 5 weeks of age showed age-related increases in thickness of the proliferative cell zone and decreases in thickness of the hypertrophic cell zone that were most apparent within the central, but not lateral, regions of the growth plate and areas of acellularity and failure of chondrocytes to transform synchronously from proliferative cell to hypertrophic cell phenotypes. Femoral ash content, composition, and accretion rates did not differ from those in normal rats during the first 5 weeks of life. These findings do not support the rachitic nature of the cartilage lesion in the tl rat. Rather, a chondrodysplastic disorder is suggested, which more closely resembles the cartilage defect present in this mutation.

摘要

患有骨质石化症的人类婴儿中常见一种看似矛盾的特征,即存在佝偻病。这种矿化紊乱在影像学和组织学上表现为生长板软骨增厚、肥大细胞区增厚以及干骺端类骨质过多,在生化方面表现为血清钙和磷浓度降低。在两种骨质石化的动物突变体,即骨硬化(oc)小鼠和无牙(tl)大鼠中也报道了佝偻病。尽管oc小鼠中佝偻病病变的表型表达与受影响人类的非常相似,但本研究结果表明tl大鼠中的病变并非如此。与正常同窝幼崽相比,对5周龄以内的tl大鼠胫骨生长板软骨进行组织学和形态计量学分析发现,增殖细胞区厚度随年龄增长而增加,肥大细胞区厚度随年龄增长而减小,这在生长板中央区域最为明显,而非外侧区域,同时还存在无细胞区以及软骨细胞未能从增殖细胞表型同步转变为肥大细胞表型的情况。在出生后的前5周内,tl大鼠股骨的灰分含量、成分和堆积速率与正常大鼠并无差异。这些发现并不支持tl大鼠软骨病变具有佝偻病性质的观点。相反,提示存在一种软骨发育异常疾病,它与该突变中存在的软骨缺陷更为相似。

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